ABIM 2015 - Rheum Flashcards
What type of JOINT pain are Cytokines (Interleukins, TNF) associated with ?
INFLAMMATORY
Redness, Heat, Swelling imply what type of JOINT pain?
INFLAMMATORY
Morning stiffness >60 minutes, fatigue, fever, malaise, joint erythema area all due to this type of JOINT condition?
INFLAMMATORY
If a joint is palpated directly and pain is elicited, what causes for the pain does that indicate?
JOINT arthritis vs injury (meniscal tear)
If the area SURROUNDING a joint is palpated and pain is elicited, what causes for the pain does that indicate?
Bursitis, Ligament strain, Tendinitis
What does PASSIVE motion of a joint mean?
Moving that joint by examiner WITHOUT ANY effort by patient
PAIN elicited on PASSIVE motion of a joint (moving that joint by examiner WITHOUT ANY effort by patient) suggests what etiology?
Arthritis
PAIN elicited on ACTIVE motion of a joint AGAINST resistance (by examiner) suggests what etiology?
Tendon pathology (overuse/excessive strain)
If an imaging study demonstrates SYMMETRIC joint-space narrowing, PERIARTICULAR osteopenia and erosions, this suggests what type of joint pathology?
Arthritis
If synovial fluid is sampled from a PAINFUL joint and demonstrates LEUKOCYTES (WBC’s) >2000/µL (mostly NEUTROPHILS), this indicates what type of JOINT pathology?
INFLAMMATORY
If an imaging study of a PAINFUL joint demonstrates ASYMMETRIC joint-space narrowing, OSTEOPHYTES and SUBCHONDRAL sclerosis, this suggests what type of joint pathology?
NON-INFLAMMATORY
Traumatic hemarthrosis or internal derangement of a joint (non-inflammatory), crystalline or septic arthritis (inflammatory) are what type of JOINT pathologies?
ACUTE MONOARTICULAR (single joint)
What must be done for ALL ACUTE MONOARTICULAR arthritis?
JOINT ASPIRATION to evaluate for INFECTION + serology ± synovial biopsy
What INFECTION sources can affect JOINTS?
Hematogenous and LOCAL skin breaks
What constitutes CHRONIC arthritis?
> 26 weeks
Mycobacteria (tuberculosis), Fungi or Borrelia burgdorferi (LYME) INFECTIONS can cause what type of arthritis?
CHRONIC MONOARTICULAR Inflammatory arthritis
What causes CHRONIC NON-INFLAMMATORY MONOARTICULAR or OLIGO-arthritis?
OSTEOARTHRITIS
Gonorrhea or Rheumatic Fever can cause what type of JOINT pathology?
ACUTE INFLAMMATORY OLIGOARTHRITIS (2-4 joints)
SPONDYLOarthritis (inflammatory rheumatic diseases) or Connective Tissue diseases can cause what type of arthritis?
CHRONIC INFLAMMATORY OLIGOARTHRITIS
When arthritis involves >4 JOINTS it may be an early manifestation of an inflammatory POLYARTHRITIS and can be caused by these infectious and autoimmune diseases?
Infections: Parvovirus B19, HIV, Hep B, Rubella
Autoimmune: RA, SLE, SPONDYLOarthritis
What do most SYSTEMIC INFLAMMATORY (RA, SLE) conditions share as a common symptom?
Fever
What do the two ARTHRITIC conditions STILL disease and Familial Mediterranean Fever share as a common symptom?
Fever
In Juvenile Inflammatory Arthritis with positive ANA, JOINTS are minimally involved, what is involved mostly?
IRITIS (iris), UVEITIS (choroid layer - posterior eye; ciliary body and iris - anterior eye)
What tests should be done in a patient who presents with otherwise ASYMPTOMATIC IRITIS (inflammation of the iris, which is part of the anterior UVEA of the eye)?
Look for SYSTEMIC inflammatory disease! CXR (r/o sarcoidosis), HLA-B27 for possible spondyloarthritis and ANCA (for granulomatosis with polyangiitis - Wegener Granulomatosis)
How many patients with RA have eye (any structure) involvement?
25% (usually keratoconjunctivitis sicca - dry eye)
Dry eyes (not keratoconjunctivitis sicca) caused by the inflammation of the LACRIMAL and other exocrine glands is seen with what SYSTEMIC inflammatory condition?
Sjögren syndrome
Rotator Cuff Tendinitis, Biceps Tendinitis, Lateral Epicondylitis, de Quervain Tenosynovitis, Carpal Tunnel Syndrome, Trigger Finger, Ischial Bursitis, Piriformis Syndrome, Trochanteric Bursitis, Pes Anserine Bursitis, Plantar Fasciitis are ALL what type of GENERAL conditions?
OVERUSE Syndromes
Tendon? Ligament?
Tendon - attachment of a muscle to bone
Ligament - attachment of bone to bone
Pain in the anterior SHOULDER when lifting heavy objects?
Biceps Tendinitis
ELBOW pain when carrying a purse/briefcase or gripping a steering wheel?
Lateral Epicondylitis
Pain in the UPPER, OUTER ARM with overhead activities or when reaching behind the back, ABDucting the arm between 30º - 150º but with less pain on PASSIVE motion of the shoulder?
Rotator Cuff Tendinitis
Pain along the RADIAL aspect of the wrist when grabbing the thumb (inside a closed fist) and stretching it with the wrist (not flexing)?
de Quervain Tenosynovitis
LOCKING of a finger on FLEXION with pain and crepitus on palpation of the tendon sheath with a flexor tendon NODULE on the PALMAR aspect of the metacarpophalangeal joint?
Trigger Finger
Pain in the buttocks worsens when sitting? With SCIATICA symptoms?
Ischial Bursitis; Piriformis Syndrome
Pain in the LATERAL HIP that worsens when lying on that side?
Trochanteric Bursitis
Pain just BELOW KNEE, anteromedially, when exercising or climbing stairs?
Pes Anserine Bursitis
Pain on the BOTTOM of the HEEL when walking?
Plantar Fasciitis
SYMMETRIC JOINT involvement, (MCP, PIP), also wrist, hips, knees, elbows, shoulders, cervical spine, dry eyes/mouth, lung disease and EROSIVE joint changes on X-ray?
RA
SYMMETRIC JOINT involvement, fever, rashes, kidney disease and neurological involvement, NO joint erosions on x-ray, POSITIVE ANA?
SLE
Ankylosing Spondylitis, Psoriatic Arthritis, Reactive Arthritis (Reiter syndrome), IBD-arthritis are ALL what type of arthritis?
HLA-B27 associated SPONDYLOarthritis
Sacroiliac and SPINAL involvement, SYMMETRIC shoulders and hips affected, SPINAL LIGAMENT CALCIFICATION on radiology, UVEITIS?
Akylosing Spondylitis (HLA-B27 positive)
ASYMMETRIC JOINT involvement, DIP joints mainly affected, sausage digits (dactylitis), EROSIONS and OSTEOPHYTES on x-rays of joints, PENCIL-in-CUP deformities, oil drop deformity in nails and nail pitting?
Psoriatic Arthritis (HLA-B27 positive)
ASYMMETRIC JOINT involvement, KNEE and ANKLES, ACHILLES Tendinitis, PLANTAR Fasciitis, SACROILIITIS, UVEITIS, KERATODERMA BLENNORRHAGICUM, CHLAMYDIA and ENTEROPATHIC infections?
Reactive Arthritis (HLA-B27 positive) - “Reiter Syndrome” - can’t see (uveitis), can’t pee (Chlamydia), can’t climb a tree (knees, ankles, sacroiliitis, plantar fasciitis, achilles tendinitis)
PAINLESS oral ulcerations (tongue and hard palate) with alopecia and painful indurated subcutaneous swelling with overlying erythema of the skin can be seen in?
SLE
Skin THICKENING involving FACE, HANDS, FEET?
Systemic Sclerosis (SCLERODERMA)
PURPURIC lesions, Cutaneous nodules, Ulcers?
Vasculitis
Oral and Genital Ulcers, Erythema Nodosum?
Beçhet disease
When you see a psoriatic-like rash on the penis “circinate balanitis,” what is it associated with?
REACTIVE Arthritis (Reiter Syndrome)
High FEVER, JOINT PAIN and a Salmon-Colored Rash on trunk and PROXIMAL extremities?
Adult-onset STILL Disease
Lots of LARGE, target/bull’s-eye lesions in association with STREPTOCOCCAL infection?
Rheumatic Fever (erythema marginatum)
What is the rash of LYME disease called?
Erythema Chronicum Migrans (or just Erythema Migrans)
Pneumonitis (bronchiolitis obliterans with organising pneumonia or interstitial lung disease) with NODULES in the lungs and pleural EFFUSIONS can be seen in this SYSTEMIC Inflammatory disease?
RA
Systemic Sclerosis (scleroderma), CREST syndrome, Lupus, granulomatosis with polyangiitis and Churg-Strauss syndrome are all systemic inflammatory diseases that can ALL affect what organ?
LUNGS
Cavitary lung NODULES with consolidation and WEDGE infarcts, hematuria with red cell casts, bloody, purulent nasal discharge are all seen in this SYSTEMIC inflammatory disease?
Granulomatosis with Polyangiitis (Wegener Granulomatosis)
Eosinophilic Pneumonitis is seen in this ASTHMA-related autoimmune vasculitis?
Churg-Strauss Syndrome
Pulmonary Artery HTN in the ABSENCE of pulmonary parenchymal disease, associated with calcinosis (calcium deposits in soft tissues), Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia is seen in?
CREST Syndrome
Diarrhea after an enteric infection (salmonella, shigella, campylobacter, yersinia, clostridium) followed by JOINT pain, uveitis is seen in?
Reactive Arthritis (Reiter Syndrome)
What two SYSTEMIC diseases is Carpal Tunnel Syndrome associated with?
DM & Hypothyroidism (but also just overuse)
Adhesive Capsulitis of the SHOULDER and Dupuytren Contracture are associated with this SYSTEMIC disease?
DM
Arthralgia and Myopathy (with elevated CK) can be seen in this thyroid condition?
HYPOthyroidism
Myopathy (with elevated CK) and Osteoporosis can be seen in this thyroid condition?
HYPERthyroidism
Calcium Pyrophosphate Deposition disease (CPPD), Hypercalcemia with Constipation and Osteoporosis can be seen in this glandular disease?
HYPERparathyroidism
Arthralgia, Bone Pain, Calcium Pyrophosphate Deposition Disease (CPDD) can be seen in this glandular disease?
Acromegaly
What disease are Hemarthroses (bleeding into joints) seen in?
Hemophilia
What does an ELEVATED ESR mean in Pregnancy, DM, Anemia and ESRD?
NOTHING, falsely elevated
What HIGHLY-ELEVATED lab value is seen in patients with Giant Cell Arteritis (temporal arteritis), Multiple Myeloma and Metastatic Cancer?
ESR >100 mm/h
What is CRP and why is it better than ESR?
It is an acute-phase reactant and a DIRECT measure of inflammation that responds more QUICKLY to changes in disease state
COMPLEMENT (C3 & C4) production and consumption increase during inflammation as it serves as a LEUKOCYTE attractant however COMPLEMENT is DECREASED (LOW) in these types of INFLAMMATORY conditions?
IMMUNE-COMPLEX MEDIATED (autoimmune) conditions - consume complement resulting in LOW complement levels (C3 and C4) - (SLE, cryoglobulinemic vasculitis, urticarial vasculitis)
Can a person with a negative ANA have lupus?
NO
Above what level ANA dilution is considered PROBABLE for lupus (IF SUSPECTED) thus warranting further studies?
ANA ≥ 1:160
What rheumatological conditions demonstrate JOINT erosions?
RA, Psoriatic Arthritis, GOUT
Positive ANA (≥1:160), Anti-ds-DNA Ab, Anti-Smith Ab are seen in what SYSTEMIC autoimmune disease?
SLE
What is the MOST SPECIFIC (rules-in disease) lab test for SLE?
Anti-Smith Ab
If positive ANA (≥1:160) and positive Anti-Smith Ab?
Almost assured to have SLE
A POSITIVE Anti-U1-RNP Ab (≥1:10,000) is seen in?
Mixed Connective Tissue Disease (MCTD)
POSITIVE Anti-Ro/SSA; Anti-La/SSB (SS stands for Sjögren Syndrome) Ab’s are seen in?
Sjögren Syndrome
POSITIVE Anti-Scl-70 and Anticentromere Ab’s are seen in what condition?
Progressive Systemic Sclerosis (scleroderma)
A POSITIVE c-ANCA is seen in what disease?
Granulomatosis with Polyangiitis (Wegener Granulomatosis)
A POSITIVE p-ANCA is seen in what disease?
Churg-Strauss Syndrome (IBD and microscopic polyangiitis)
A POSITIVE Anti-Jo-1 Ab is seen in what disease?
Myositis
What is the MOST SENSITIVE (rules-out disease) lab test for RA?
Anti-cyclic citrullinated peptide (NO, definitely NOT RF)
Antihistone Ab’s are seen in what condition?
DRUG-induced SLE
What diseases are CRYOGLOBULINS seen in?
HEP C, also in Vasculitis and Multiple Myeloma
What is the use of plain radiographs in monitoring OSTEOARTHRITIS?
Evaluating the severity of Joint Space Loss
When evaluating the EROSIVE lesions of JOINTS in diseases such as RA, Psoriatic Arthritis or GOUT, what imaging is REQUIRED?
TWO (2) VIEW Plain Radiographs
What is the BEST imaging modality for BONE if plain radiographs are not sufficient?
CT
What is the BEST imaging modality for SOFT TISSUES (cartilage, synovium, tendons, ligaments)?
MRI
What test is used to differentiate between INFLAMMATORY and NON-inflammatory JOINT effusions, ESPECIALLY when distinguishing between infectious arthritis and acute crystalline arthropathy?
JOINT Aspiration (Cell count, Gram stain w/culture, Crystal analysis)
Fever, Leukocytosis (elevated WBC’s), elevated INFLAMMATORY markers are POSITIVE in both Infectious Arthritis and Gout, how can you distinguish between the two?
JOINT Aspiration (Cell count, Gram stain w/culture, Crystal analysis)
A JOINT Aspirate with ≥50,000/µL leukocytes (mostly PMN’s) is suspicious for what?
INFECTIOUS Arthritis
A JOINT Aspirate with ≤2,000/µL leukocytes NOT associated with what?
NOT associated with an INFLAMMATORY condition
Does the presence of CRYSTALS in a JOINT aspirate rule out INFECTION?
NO (not if suspicion is high)
NEEDLE shaped, NEGATIVELY-birefringent, yellow/blue crystals in JOINT aspirate indicate what?
GOUT (urate crystals)
RHOMBOID shaped, POSITIVELY-birefringent, yellow/blue crystals in JOINT aspirate indicate what?
Calcium Pyrophosphate Crystals (CPPD) - PSEUDOgout
Bony EROSIONS, Joint Subluxations, MCP and PIP HAND joint involvement are seen in?
RA
Bony EROSIONS and OSTEOPHYTES, DIP involvement, PENCIL-in-CUP deformities, oil-drop nails and nail-pitting are seen in?
Psoriatic Arthritis
ASYMMETRIC JOINT-SPACE Narrowing, Osteophytes, CYSTIC changes, collapse of spinal disks, DIP (Heberden nodes) and PIP (Bouchard nodes) involvement, kyphosis are seen in?
Osteoarthritis
Sacroiliitis with SQUARING of the vertebral bodies are seen in?
Ankylosing Spondylitis
Calcification of a spinal ligament (anterior longitudinal ligament) is seen in?
Diffuse Idiopathic Skeletal Hyperostosis (DISH)
Chondrocalcinosis of the KNEES, SHOULDERS, WRISTS and PUBIC SYMPHYSIS is seen in?
Calcium Pyrophosphate Deposition disease (CPPD) - PSEUDOgout - yellow/blue, RHOMBOID, positively-birefringent crystals
What are the Disease Activity Score (DAS) and the Health Assessment Questionnaire?
Instruments used to measure Rheumatic disease activity and progression as well as overall patient function and wellness
These agents inhibit cyclooxygenase (COX) enzymes and block prostaglandin E2 generation?
NSAIDS (reducing prostaglandin E2 provides most of the anti-inflammatory and analgesic effects of NSAIDS however prostaglandin E2 maintains the gastric lining and kidney blood flow
These NSAIDS cause LESS GI adverse effects and less asthma effects but do not alter renal toxicity or HTN?
COX-2 (selective) inhibitors - CELECOXIB
Which is the ONLY NSAID that DOESN’T cause cardiovascular risk?
Naproxen
What are two good analgesic-only medications that are not anti-inflammatory and not addicting?
Acetaminophen and Tramadol
The drug is used in the treatment of GOUT, PSEUDOgout and Familial Mediterranean Fever?
Colchicine
What should be given to all patients on chronic corticosteroid therapy?
Calcium + Vitamin D and bisphopshonates (to avoid osteoporosis)
What are DMARDS (Disease Modifying Anti-Rheumatic Drugs)?
IMMUNOSUPPRESSIVE agents
What is the GOLD-standard DMARD for RA?
Methotrexate (also used for Psoriatic Arthritis, Systemic Vasculitis, Polymyositis)
LIVER toxicity and macrocytic ANEMIA (interferes with liver hematopoiesis) are two adverse effects of this DMARD used for RA requiring routine surveillance blood work and PROHIBITING ALCOHOL use during treatment?
Methotrexate (folic acid antagonist) - EXTREMELY TERATOGENIC
What DMARD used for the treatment of RA can CAUSE multiple RA nodules (NODULOSIS) and pneumonitis?
Methotrexate (folic acid antagonist) - EXTREMELY TERATOGENIC
What can be added to METHOTREXATE to reduce its toxicity but not affect its therapeutic effects?
Folic Acid
Because METHOTREXATE is a HIGHLY TERATOGENIC drug, WHEN must it be discontinued prior to pregnancy?
AT LEAST 3 MONTHS PRIOR to CONCEPTION!!!
This DMARD is used frequently in the treatment of LUPUS to prevent flares and reduce morbidity and mortality?
Hydroxychloroquine (pregnancy SAFE)
What DMARD can cause visual loss due to RETINAL PIGMENT deposition requiring routine eye exams?
Hydroxychloroquine (pregnancy SAFE)
This DMARD is used mainly for the treatment of IBD however it does have moderate effect in RA and can cause agranulocytosis, hepatitis and reversible oligospermia?
Sulfasalazine (pregnancy SAFE)
This DMARD is as good as METHOTREXATE (gold-standard for RA treatment) with adverse effects of interstitial ling disease (like methotrexate) and like methotrexate, it is EXTREMELY teratogenic?
Leflunomide (EXTREMELY TERATOGENIC)
What must be done which is UNIQUE to this DMARD when planning pregnancy due to its EXTREMELY TERATOGENIC effects and EXTREMELY LONG half-life (MONTHS)?
Leflunomide (EXTREMELY TERATOGENIC) - MUST take cholestyramine TID x 8 days to remove from system and recheck levels TWICE prior to conception
This DMARD is a corticosteroid-SPARING agent and also used in maintenance of LUPUS, IBD, Vasculitis and Polymyositis?
Azathioprine (TPMT) - pregnancy SAFE
This DMARD, a potent ALKYLATING agent is used to treat life-threatening LUPUS and systemic VASCULITIS however can cause BLADDER Cancer, LYMPHOMA and Hemorrhagic Cystitis requiring close blood work monitoring?
Cyclophosphamide (NOT safe for pregnancy - D)
When is the ONLY time pregnancy class D DMARD Cyclophosphamide should be used in a pregnant patient?
When the LIFE is in DANGER
This Anti-REJECTION DMARD inhibits BOTH B-lymphocytes and T-lymphocytes and is used to treat LUPUS NEPHRITIS and is used as a corticosteroid-SPARING agent for systemic Vasculitis and Polymyositis while having fewer side effects than Cyclophosphamide?
Mycophenolate Mofetil (TERATOGENIC)
This DMARD specifically targets T-lymphocytes and is used in MANY rheumatic and autoimmune disease with side effects such as HIRSUTISM, NEPHROTOXICITY and TREMOR) therefore used only as a THIRD-line agent?
Cyclosporine (pregnancy SAFE)
These class of DMARDS have a much greater SPECIFICITY for targeted immune suppression with fewer side effects?
BIOLOGIC AGENTS (TNF-α, Anti-IL, anti-CD20, etc.)
What is necessary prior to use of ALL biologic agents?
Latent TUBERCULOSIS test (PPD or interferon-γ release assay)
What VACCINATION limitations exist for patients using biologic agents?
NO LIVE VACCINES; patients taking either RITUXIMAB or ABATACEPT should get ALL immunizations done PRIOR to use of these agents (live or killed vaccines)
What other DMARD should anti-TNF-α agents be used in COMBINATION with for BEST RA treatment?
METHOTREXATE (folic acid antagonist) or LEFLUNOMIDE - both EXTREMELY TERATOGENIC
What should be done if a patient is taking an anti-TNF-α medication and develops an infection?
anti-TNF-α drug should be stopped and the infection TREATED prior to restarting
Reactivation of TUBERCULOSIS, HISTOPLASMOSIS and COCCIDIOIDOMYCOSIS and He B are all associated with these agents?
anti-TNF-α drugs (pregnancy SAFE)
What should be done when a patient taking anti-TNF-α drugs develops psoriaform skin eruptions, demyelinating syndromes or drug-induced lupus?
STOP the anti-TNF-α drug
What two cancers have been associated with anti-TNF-α drugs and their development requires prompt discontinuation of the drug?
LYMPHOMA and non-melanoma skin cancers
A patient who has moderate or high autoimmune disease activity and POOR PROGNOSIS and has FAILED METHOTREXATE/LEFLUNOMIDE or METHOTREXATE/LEFLUNOMIDE in combination with other DMARDS or FAILED anti-TNF-α drugs can be started on this IMMUNOSUPPRESSIVE medication that can cause LUNG CANCER, COPD exacerbations and LYMPHOMA?
Abatacept (pregnancy SAFE)
What BIOLOGIC agent that antagonizes IL-1ß receptors is used to treat adult-onset STILL disease (HIGH fevers, JOINT pain, Salmon colored rash on trunk and PROXIMAL extremities)?
Anakinra (pregnancy SAFE)
How does RITUXIMAB work and why is it used in many of the LYMPHOMAS and LEUKOCYTIC leukemias?
RAPIDLY depletes anti-CD20 (B-cell) lymphocytes
In patients with RA who have NOT responded well to the COMBINATION of METHOTREXATE/LEFLUNOMIDE + anti-TNF-α drug, what other medication can be used? (this drug has also been shown to be AS EFFECTIVE as Cyclophosphamide in inducing REMISSION in patients with ANCA-associated VASCUITIS)
RITUXIMAB (anti-CD20 “B-cell”) - pregnancy SAFE
Severe INFUSION reactions and RARE reactivation of the JC-virus causing PML have been seen with this drug?
RITUXIMAB (pregnancy SAFE)
In patients with RA who have NOT responded well to an anti-TNF-α drug, what anti-IL-6 receptor antagonist medication can be used that can cause gastric/intestinal RUPTURE and increased SERUM LIPID levels?
Tocilizumab (pregnancy SAFE)
A BIOLOGIC drug used for Crohn disease, Psoriasis, Psoriatic Arthritis and RA that targets TNF-α BEFORE it is formed by antagonizing IL-12 and IL-23 via the p40 subunit is called?
Ustekinumab (pregnancy SAFE)
What BIOLOGIC agent targets B-cell lymphocytes BEFORE they are fully formed and therefore works well specifically for SLE and Sjögren syndrome?
Belimumab (pregnancy SAFE)
What is the MOST EFFECTIVE therapy for RA?
COMBINATION of Methotrexate/Leflunomide + anti-TNF-α inhibitors
A patient who experiences ≥2 gout attacks/YEAR or has gouty urate deposits (TOPHI) requires what?
GOUT treatment
Why is colchicine (NSAID) required when initiating GOUT therapy with a maintenance GOUT agent (allopurinol, febuxostat, probenecid)?
Because initiating a maintenance GOUT medication without the COLCHICINE (NSAID) can trigger GOUT attacks for 3-6 MONTHS
What is the most common side effect of ALLOPURINOL for which it MUST be discontinued?
RASH (can cause Stevens-Johnson syndrome)
Which xanthine oxidase inhibitor is used in patients with mild-to-moderate kidney failure?
FEBUXOSTAT (not a purine analog like allopurinol)
What GOUT drug requires EXCELLENT kidney function and DOES NOT work in patients with KIDNEY DISEASE and can cause KIDNEY STONES which is why ALL patients using this medication must keep VERY WELL HYDRATED?
Probenecid
What medication is used to treat the very high concentration of URIC ACID seen in TUMOR LYSIS SYNDROME, however cannot be used to treat GOUT chronically because it is HIGHLY IMMUNOGENIC?
RASBURICASE
If standard medications fail to control GOUT, what ONE medication can be used, which is a PEG-form of rasburicase?
PEGloticase
White women, between the ages of 30-55 can develop a chronic SYSTEMIC INFLAMMATORY disease with JOINT destruction that increases their prevalence of CAD?
Rheumatoid Arthritis (RA)
A largely inherited condition in which TNF-α induced OSTEOCLAST activation and production of degradative enzymes leads to cartilage damage and bone erosion?
Rheumatoid Arthritis (RA)
How does pregnancy and breast-feeding affect RA?
Risk of RA is reduced in women who have had children and breast-fed for at least 1 year and RA subsides during pregnancy and flares after
The presence of BOTH RF and anti-cyclic citrullinated peptide Ab’s in the blood suggests what?
RA
What preventable risk factor is STRONGLY associated with RA?
Smoking
Prolonged >60 min MORNING STIFFNESS, SYMMETRIC POLYARTHRITIS of small, medium and large joints involving the MCP’s and PIP’s of the hands, the wrists with deformity and joint dysfunction but SPARING the DIP’s and LUMBAR spine suggest what diagnosis?
Rheumatoid Arthritis (RA)
Anemia of CHRONIC disease, elevated ESR, CRP and ANA (40%), thrombocytosis and HYPOalbuminemia, JOINT aspirates demonstrate elevated leukocyte counts with neutrophil predominance, positive RF(70%) - also seen in MIXED Cryoglobulinemia, Sjögren syndrome and SLE - and positive anti-cyclic citrullinated Ab’s (60%) all suggest what?
Rheumatid Arthritis (RA)
Does the ABSENCE of BOTH RF and anti-cyclic citrullinated Ab’s effectively rule out Rheumatoid Arthritis (RA)?
NO!!
CXR demonstrating periarticular OSTEOPENIA, SYMMETRIC JOINT-SPACE NARROWING and EROSIONS (at the margins of joints) are findings that are often seen in?
Rheumatoid Arthritis (RA)
What imaging modality can detect BONE EROSIONS EARLIER than CXR?
MRI (bone marrow edema and synovial proliferation)
Ulnar deviation of phalanges, Swan neck and Boutonnière deformity, popliteal (Baker) and ganglion cysts are seen in?
Rheumatoid Arthritis (RA)
What is a syndrome of RA where a patient has PANcytopenia, Splenomegaly and LEG ULCERS?
Felty syndrome
CAD, Felty syndrome, vasculitis, nodules, interstitial lung disease, scleritis with scleral ulceration (scleral melt) and secondary amyloidosis are all manifestations seen in what disease?
Rheumatoid Arthritis (RA)
What specific treatment for RA also results in significant reduction of cardiovascular risk?
Methotrexate/Leflunomide + anti-TNF-α inhibitor
What constitutes INSUFFICIENT therapeutic regiment for RA?
Progressive JOINT damage on plain radiographs
LEG ULCERS in RA are part of what RA-syndrome?
Felty syndrome
What is the BEST INITIAL MONOtherapy for a patient with RA of ANY duration or disease activity?
Methotrexate
What patients with RA would be good candidates for initial MONOtherapy with HYDROXYCHLOROQUINE, SULFASALAZINE or MINOCYCLINE?
Those who have had RA for a short time and with a LOW disease activity
What type of therapy can be used for patients with RA that have POOR prognostic features or moderate-to-high disease activity levels?
COMBINATION therapy using METHOTREXATE with HYDROXYCHLOROQUINE or SULFASALAZINE OR any of these with a BIOLOGIC agent
Which of the BIOLOGIC agents used to treat RA tends to provide a better response in patients with POSITIVE Rheumatoid Factor (RF)?
RITUXIMAB
What can be done for a patient with RA that has failed MONOtherapy, COMBINATION therapy with NON-biologic DMARD’s AND COMBINATION therapy with a BIOLOGIC DMARD?
Change the BIOLOGIC DMARD to another (usually after the second change, pt is LESS likely to respond to a third, etc)
What specific RISK in a patient with RA must be checked for in the PRE-OP management as missing this can result in potential CORD COMPRESSION during intubation?
Atlanto-Axial Subluxation (evaluate the cervical spine with BOTH flexion AND extension RADIOGRAPHS
What should be done for a patient with ANEMIA of CHRONIC DISEASE after surgery with significant BLOOD LOSS?
TRANSFUSE
What RA meds should be stopped prior to elective SURGERY?
NSAIDS (to reduce bleeding risk) and anti-TNF-α inhibitors
What two vaccines should be given to ALL patients taking immunosuppressive agents whether diagnosed with RA or any other disease?
INACTIVE Pneumococcal and Influenza Vaccines
Are LIVE-attenuated vaccines safe for use in patients with RA taking BIOLOGIC medications?
NO!!
Can LIVE-attenuated vaccines be used in patients taking NON-BIOLOGIC DMARD’s ± Steroids (prednisone, etc.)?
YES (if low-dose for BOTH)
What should MEN do if they are taking SULFASALAZINE for RA and planing to have a family? Why?
Discontinue it 3 MONTHS before
Because SULFASALAZINE causes OLIGOSPERMIA (reduced sperm-count)
What can NSAIDS cause if taken early during pregnancy?
Interfere with IMPLANTATION
What can NSAIDS cause if taken during the THIRD TRIMESTER?
PREMATURE closure of the DUCTUS ARTERIOSUS
What can CORTICOSTEROIDS cause if taken BEFORE 14 weeks gestation?
CLEFT PALATE (fetus), Gestational Diabetes and HTN (mother)
Are NSAIDS, LOW-dose PREDNISONE or METHOTREXATE safe to use during lactation?
NSAIDS and LOW-dose PREDNISONE - YES
METHOTREXATE - NO!!
What is the MOST COMMON condition affecting 80% of patients ≥55 and 95% of patients ≥65?
OSTEOARTHRITIS (biomechanical rather than immunological process)
Acute JOINT injury (ligament tear) or repetitive (overuse/obesity) can result in JOINT laxity, cartilage injury, abnormal joint mechanics that can all lead secondarily to what condition?
OSTEOARTHRITIS (biomechanical rather than immunological process)
What is the SINGLE most MODIFIABLE risk factor for OSTEOARTHRITIS of the KNEE?
OBESITY
Morning stiffness
OSTEOARTHRITIS (biomechanical rather than immunological process)
Bouchard (DIP) and Heberden (PIP) nodes as well as bone spurs are seen in this disease?
OSTEOARTHRITIS (biomechanical rather than immunological process)
Involvement of the FIRST CarpoMetaCarpal (CRC) JOINT of the hand (base of thumb) causes “SQUARING” of the wrist in this disease?
OSTEOARTHRITIS (biomechanical rather than immunological process)
ASYMMETRIC JOINT-SPACE narrowing resulting in a VALGUS (lateral joint space - knocked knee - MOST COMMON) or VARUS (medial joint space - bow-legged) deformities are seen in what condition?
OSTEOARTHRITIS (biomechanical rather than immunological process)
Loss of spinal mobility with CANAL STENOSIS due to bone SPURS or DISK DEGENERATION (bulging, fragmentation) leading to NERVE root impingement (cervical radiculopathy, SCIATICA, pseudoclaudication - from lumbar spinal stenosis) or MYELOPATHY (from cervical spinal stenosis) can be seen in?
OSTEOARTHRITIS (biomechanical rather than immunological process)
HAND joints (PIP, DIP, CMC), HIP and KNEE joints are affected in what disease?
OSTEOARTHRITIS (biomechanical rather than immunological process)
When an ATYPICAL JOINT is involved in OSTEOARTHRITIS (typically involved joints: PIP, DIP, CMC, HIP and KNEE), what is suspected to be the cause?
Chondrocalcinosis, Acromegaly or Hemochromatosis OR due to PREVIOUS joint injury due to RA or INFECTION
This erosive disease typically involves ONLY the HAND, it is seen mostly in WOMEN, with FLARES of INFLAMMATION of the DIP and PIP joints with ERYTHEMA, SWELLING, SEVERE PAIN and plain films demonstrating erosions and spurs?
EROSIVE OSTEOARTHRITIS
Calcification of the THORACIC SPINAL LIGAMENTS with little disk narrowing, associated with Achilles or Calcaneal Spurs and calcification of the QUADRICEPS muscle in OBESE MEN ≥40 with symptoms of stiffness and reduced range of motion and elevated levels of INSULIN-like Growth Factor-1 and GROWTH HORMNE?
Diffuse Idiopathic Skeletal Hyperostosis (DISH)
Te demonstration on X-rays of “FLOWING Osteophytes Across FOUR contiguous vertebrae” provides the diagnosis of what?
Diffuse Idiopathic Skeletal Hyperostosis (DISH)
What should be suspected in patients aged ≥40 who have CHRONIC JOINT PAIN WITHOUT significant MORNING STIFFNESS (DIP, PIP, CMC, HIP, KNEE, SPINE)?
OSTEOARTHRITIS (biomechanical rather than immunological process)
Do you see an elevated ESR, CRP or significant Morning Stiffness in a patient with OSTEOARTHRITIS?
NO
A patient presenting with JOINT pain that is described as GROIN pain or BUTTOCK pain worsened by WEIGHT BEARING likely has what?
OSTEOARTHRITIS of the HIP (seen on X-rays as joint space narrowing)
Pain that is WORSENED when STANDING or WALKING suggests osteoarthritis of what?
The KNEE
Pain that is WORSENED when CLIMBING or DESCENDING STAIRS or when SITTING for a PROLONGED period of time in a patient with osteoarthritis likely involves what joint?
The Patello-Femoral joint
BONE CYST formation, JOINT-SPACE narrowing and OSTEOPHYTES can ALL be seen in this condition?
OSTEOARTHRITIS
What are the preferred, NON-PHARMACOLOGIC treatments for patients with OSTEOARTHRITIS?
COMBINATION of WEIGHT LOSS, EXERCISE (physical therapy) and unloading braces (hip and knee)
How is a CANE properly used as an UNLOADING device in a patient with KNEE or HIP OSTEOARTHRITIS?
It is held in the CONTRALATERAL HAND of the affected JOINT and SWUNG forward with the SWINGING of the AFFECTED limb
In addition to NON-PHARMACOLOGIC agents, which PHARMACOLOGIC agent should be used as FIRST-LINE therapy in a patient with OSTEOARTHRITIS?
ACETAMINOPHEN (then NSAID’s)
What medications can be used for maintenance of function and alleviating pain in patients with OSTEOARTHRITIS refractory to combinations of non-pharmacologic therapy with acetaminophen or NSAID’s?
Lidocaine patches (KNEE) or low-dose OPIOIDS
What can be done for patients with OSTEOARTHRITIS who have ONE JOINT that is much more symptomatic with PAIN and loss of function than the rest?
INTRA-ARTICULAR corticosteroid injection (lasts 3 MONTHS)
What are some of the adverse effects seen when patients with OSTEOARTHRITIS receive >3 INTRA-ARTICULAR corticosteroid injections for symptom relief and restoration of function?
Cartilage atrophy, Fat atrophy and skin Depigmentation
An alternative to INTRA-ARTICULAR corticosteroid injections for symptom control and restoration of function for patients with OSTEOARTHRITIS that lasts 3-6 MONTHS (3 months with corticosteroids), is most efficacious if injected AFTER the joint EFFUSION is drained and there is NO LIMIT to REPEAT injections however is more expensive?
HYALURONIC ACID Injections
In patients with ADVANCED OSTEOARTHRITIS with SIGNIFICANT symptoms of PAIN and LOSS of FUNCTION that affect quality of life and are not responsive to exercise, weight loss and medical therapy, what is the MOST effective SURGICAL treatment?
JOINT REPLACEMENT SURGERY ‘arthroplasty” (HIP, KNEE) with VIGOROUS post-OP REHABILITATION
A CENTRAL pain sensitivity syndrome associated with physical and emotional stressors with WIDE-SPREAD pain and tenderness with ALLODYNIA (pain due to a stimulus that does NOT typically produce pain) and hyperalgesia?
FIBROMYALGIA
First-Degree relatives of patients with this condition are more likely to have MOOD disorders and MIGRAINES?
FIBROMYALGIA
MOOD disorders, FATIGUE, MYALGIA, ARTHRALGIA, PARESTHESIAS, SLEEP DISTURBANCE and COGNITIVE DYSFUNCTION are commonly associated with this disorder?
FIBROMYALGIA
Aside from PAIN, are there any abnormalities noted on physical examination, routine laboratory or imaging tests in patients with FIBROMYALGIA?
NO
WIDE-SPREAD pain, NOT “tenderness” (R & L sides of the body, ABOVE & BELOW the waist, pain in the spine especially LOW BACK PAIN and SHOULDER and BUTTOCK pain) in 11 of 18 SITES elicited on DIGITAL examination is diagnostic for what?
FIBROMYALGIA
Besides treating the underlying issues in a patient with FIBROMYALGIA (sleep, mood disorders, etc.), what is the BEST FIRST-LINE treatment?
NON-PHARMACOLOGIC (REGULAR Aerobic EXERCISE for 30 min/day) with PHYSICAL and COGNITIVE BEHAVIORAL therapy
What are the BEST pharmacologic choices for the treatment of FIBROMYALGIA?
SNRI’s (DULOXETINE or MILNACIPRAN), PREGABALIN or GABAPENTIN and TCA’s (amitriptyline), Cyclobenzaprine
What are the INFLAMMATORY disorders Ankylosing Spondylitis, Psoriatic Arthritis, IBD-Associated Arthritis and Reactive Arthritis (Reiter Syndrome) with inflammation and CALCIFICATION of ENTHESES (the connections between tendons/ligaments and BONE) and NEW BONE formation part of?
SPONDYLOARTHRITIS (HLA-B27 associated)
Family history and HLA-B27 positivity are STRONGLY associated with what disease cluster?
SPONDYLOARTHRITIS
What are the common triggers for REACTIVE Arthritis, a SPONDYLOARTHRITIS?
GI and Urinary infections
What is the trigger commonly seen for PSORIATIC ARTHRITIS, a SPONDYLOARTHRITIS?
Recent TRAUMA/Repeated microTRAUMA (knees and elbows)
What is thought to be the trigger for IBD-associated Arthritis, a SPONDYLOARTHRITIS?
Enteric Bacteria
What should you test for in a patient with PSORIATIC ARTHRITIS or REACTIVE ARTHRITIS who develops a SUDDEN ONSET or SUDDEN INCREASED SEVERITY of their condition?
HIV
In what two (2) SPONDYLOARTHRITIS conditions can you see SAUSAGE-SHAPED digits?
Psoriatic Arthritis and Reactive Arthritis (due to dactylitis)
AXIAL and PERIPHERAL arthritis, Enthesitis, Dactylitis as well as Gastrointestinal, Dermatologic and Ophthalmologic INFLAMMATION is seen in this cluster of disease?
SPONDYLOARTHRITIS
What does the word “SPONDYLO-“ mean?
Affecting the SPINAL vertebra
A MALE patient aged 20-30 presents with c/o PROGRESSIVE INFLAMMATORY BACK PAIN and STIFFNESS of the low back, buttocks, posterior thighs which in time, ASCENDS up the SPINE producing a STOOPED posture with limited mobility of the SPINE and CHEST?
SPONDYLOARTHRITIS
EVEN in a history WITHOUT TRAUMA, back pain in a patient with SPONDYLOARTHRITIS that is NEW, WORSENING or DIFFERENT in character should be examined how and for what?
SPINE Imaging to evaluate for VERTEBRAL fracture, MYELOPATHY and RADICULOPATHY
Progressive ASCENING PAIN and STIFFNESS in the LOWER BACK, BUTTOCKS, POSTERIOR THIGHS and involving the SACROILIAC JOINTS, AORTITIS, RESTRICTIVE LUNG DISEASE, ASYMPTOMATIC INTESTINAL ULCERS and UNILATERAL ANTERIOR UVEITIS is associated with what SPONDYLOARTHRITIS?
Ankylosing Spondylitis
PSORIATIC ARTHRITIS, a SPONDYLOARTHRITIS, is more commonly associated with what OPHTHALOMOLOGIC feature?
CONJUNCTIVITIS
PYODERMA GANGRENOSUM and ERYTHEMA NODOSUM are dermatologic manifestations most commonly seen with what SPONDYLOARTHRITIS that also is associated with UVEITIS, NEPHROLITHIASIS, Vitamin D Deficiency and THROMBOEMBOLISM?
IBD-associated arthritis
KERATODERMA BLENORRHAGICUM and CIRCINATE BALANITIS, CONJUNCTIVITIS are associated with this SPONDYLOARTHRITIS disease and often triggered by a GI or GU infection?
Reactive Arthritis
What secondary, painful disease can occur in PSORIASIS due to its high cell-turnover?
GOUT
RF and anti-cyclic citrullinated peptide Ab’s are NOT usually present in pt’s with PSORIATIC ARTHRITIS (a spondyloarthritis). What does it indicate when a patient with PSORIATIC ARTHRITIS is found to be positive for anti-cyclic citrullinated peptide Ab’s?
EROSIVE disease
What does the COEXISTENCE of EROSIVE or LYTIC changes with NEW BONE FORMATION at the ENTHESES signify?
PSORIATIC ARTHRITIS
What two joints are MAINLY affected in IBD-associated arthritis and can be infected?
KNEE and MCP
A post-infectious (Chlamydia, Yersinia, Salmonella, Shigella, Campylobacter, Clostridium) aseptic arthritis (a spondyloarthritis) that often presents with urethritis and conjunctivitis?
REACTIVE ARTHRITIS
Oral ulcers, genital lesions (circinate balanitis), nail changes as seen with psoriatic arthritis and rashes (keratoderma blenorrhagicum) are associated with this spondyloarthritis?
REACTIVE ARTHRITIS
What should be considered in a patient
SPONDYLOATHRITIS
Tenderness at the calcaneal attachments of the Achilles tendon, plantar fascia or costochondral junctions with improvement when treated with NSAIDS suggests what?
ENTHESITIS (spondyloarthrtitis)
What two diseases should be checked for in young patients who develop Reactive Arthritis or Psoriatic Arthritis with SEVERE skin manifestations?
HIV and Chlamydia Trachomatis
In a patient where SPONDYLOARTHRITIS is highly suspected from symptoms of inflammation at the sacroiliac JOINTS but plain radiographs fail to show sacroiliitis, what testing modality should be done next?
MRI to look for BONE MARROW edema and detection of ACTIVE INFLAMMATION
What is the FIRST-LINE therapy for the treatment of SPNDYLOARTHRITIS?
NSAIDS (both for AXIAL and PERIPHERAL involvement)
WHEN would you use DMARD’s for the treatment of SPONDYLOARTHRITIS?
ONLY when treating PERIPHERAL (not axial) involvement
What can be done to treat an affected JOINT acutely in SPONDYLOARTHRITIS as NSAIDS take effect?
INTRA-ARTICULAR Injection of CORTICOSTEROIDS
If symptoms are not adequately controlled in patients with SPONDYLOARTHRITIS with NSAIDS and intra-articular injections of corticosteroids, what other medication will they respond to?
Anti-TNF-α inhibitors
What can be done to treat CERVICAL INSTABILITY and DEFORMITY in patients with SPONDYLOARTHRITIS?
SURGERY (fusion, wedge osteotomy)
What type of treatment should be avoided in patients with PSORIASIS because they can cause a FALRE of SKIN manifestations?
SYSTEMIC CORTICOSTEROIDS
What should PERIPHERAL manifestations of SPONDYLOARTHRITIS be treated with if NSAIDS and corticosteroids are not sufficiently effective in treating moderate to severe inflammation?
NON-BIOLOGIC DMARD’s (methotrexate, sulfasalazine, cyclosporine, leflunomide)
Do DMARD’s inhibit radiologic progression of JOINT inflammation and erosion in patients with SPONDYLOARTHRITIS?
NO
What medications are MORE effective than NON-BIOLOGIC DMARD’s and HAVE been shown to inhibit radiologic progression of JOINT inflammation and erosion in SPONDYLOARTHRITIS?
BIOLOGICS (anti-TNF-α inhibitors)
When should BIOLOGIC agents be used as a FIRST-LINE treatment for patients with SPONDYLOARTHRITIS?
When POOR prognostic factors such as polyarticular inflammation, joint damage and impaired function/poor quality of life exist
In what SPONDYLOARTHRTIS disease does the COMBINATION of anti-TNF-α inhibitors with METHOTREXATE not provide added benefit?
Ankylosing Spondylitis
A systemic inflammatory disease, most commonly affecting BLACK WOMEN of REPRODUCTIVE AGE thought to be triggered by viruses and other environmental exposures in a genetically-susceptible person?
SLE
PHOTOSENSITIVE rashes, ALOPECIA, MUCOSAL ULCERS on the HARD PALATE, Tongue and Buccal Mucosa?
SLE
Which of the SLE rashes causes IRREVERSIBLE ALOPECIA?
DISCOID LUPUS RASH
Immunoglobulin (IgG) and COMPLEMENT deposits at the DERMAL-EPIDERMAL JUNCTION?
POSITIVE LUPUS BAND TEST
What JOINTS are commonly affected (arthralgia and arthritis) in SLE?
HANDS, WRISTS, KNEES
HAND tendon inflammation in this disease mimics the appearance of RA however is not destructive to the joint and is reversible, called Jaccoud arthropathy?
SLE
PROXIMAL muscle weakness with normal CK indicates what?
CORTICOSTEROID-INDUCED Myopathy
CHRONIC GENERALIZED MYALGIA with normal CK indicates what?
FIBROMYALGIA
Avoidance of the sun, decreased physical activity and chronic corticosteroid use in SLE causes this condition?
OSTEOPOROSIS
PROTEINURIA (3+ or >0.5 g/day), HEMATURIA and CELLULAR CASTS with HTN, EDEMA and Kidney Failure - NEPHRITIS that occurs mostly in BLACKS with this systemic inflammatory disease?
SLE (PROLIFERATIVE NEPHRITIS)
How is LUPUS NEPHRITIS (PROLIFERATIVE NEPHRITIS) diagnosed?
Kidney BIOPSY
How is LUPUS NEPHRITIS (PROLIFERATIVE NEPHRITIS) initially treated upon SUSPICION?
URGENTLY!! with CORTICOSTEROIDS (no need to await biopsy results)
What is the definitive treatment for LUPUS NEPHRITIS (PROLIFERATIVE NEPHRITIS)?
CORTICOSTEROIDS + either cyclophosphamide or mycophenolate mofetil
What do the presence of CELLULAR CRESCENTS and INTERSTITIAL FIBROSIS suggest in a Kidney BIOPSY?
POOR KIDNEY PROGNOSIS
Aseptic Meningitis, Cerebrovascular Disease (stroke), Demyelinating Syndrome, Chorea, Seizures, Myelopathy Cognitive Dysfunction, Anxiety, HA, Mood Disorder and Psychosis can all be seen in this systemic inflammatory disease?
SLE (Neuro-Psychiatric manifestations of SLE)
What is the MOST FREQUENT Cardiac manifestation of SLE?
PERICARDITIS
The presence of ANTIPHOSPHOLIPID AB’s in SLE can result in what CARDIAC manifestation?
VALVULAR Heart Disease
What is Libman-Sacks Endocarditis?
Asymptomatic Sterile Vegetations seen in SLE that can lead to embolization and superimposed infectious endocarditis
What should be done for ANY patient with SLE who presents with complaints of CHEST PAIN and why?
ECG, because of accelerated atherosclerosis seen in systemic inflammatory diseases such as SLE
What is the MOST COMMON Pulmonary manifestation of SLE?
PLEURITIS
Acute Pneumonitis, Interstitial Lung Disease, Diffuse Alveolar Hemorrhage (deadly), Shrinking Lung Syndrome, PE and Pulmonary HTN are seen in this systemic inflammatory disease?
SLE
Thrombocytopenia, Coomb’s POSITIVE hemolytic anemia, anemia of chronic disease and anemia due to kidney failure are all seen in this systemic inflammatory disease?
SLE
What specific ANEMIA type can often be the FIRST symptom of SLE?
Idiopathic Thrombocytopenic Purpura
Anticardiolipin Ab’s, anti-ß2-glycoprotein I antibodies and Lupus Anticoagulant are called what?
Antiphospholipid Ab’s, found in 35% of patients with SLE
Arterial or Venous Thrombosis, Pregnancy Loss or Complications, Livedo Reticularis, Thrombocytopenia are caused by what in SLE?
The presence of Antiphospholipid Ab’s
How is PRIMARY Antiphospholipid Syndrome treated?
ANTICOAGULATION
How is Antiphospholipid Syndrome treated when part of SLE?
Immunosuppressive Therapy + Anticoagulation
What malignancies are higher risk in patients with SLE?
LYMPHOMA, LUNG and HEPATOBILIARY Cancer
Does the ANA titer fluctuate with the level of autoimmune disease activity?
NO
Positive anti-DS-DNA Ab’s, anti-Smith Ab’s and LOW C3, C4 and CH50 are HIGHLY suggestive of?
SLE
What virus can cause JOINT pain and transiently-positive autoimmune serologies as seen with SLE?
Parvovirus-B19
Anti-Single-Stranded DNA Ab’s, Anti-Histone Ab’s, normal COMPLEMENT levels seen in patients taking PROCAINAMIDE, HYDRALAZINE, INH, METHYLDOPA, MINOCYCLINE and anti-TNF-α inhibitors which resolves within 6 WEEKS after discontinuation of DRUGS?
DRUG-Induced Lupus
What is a SENSITIVE (but not specific), meaning it can rule-out but not rule-in, screening test for SLE?
ANA
What are the two SPECIFIC (rule-in) lab tests available for SLE?
Anti-DS-DNA Ab’s (fluctuates with disease activity prevalence is 70%) and Anti-Smith Ab’s (does not fluctuate with disease activity, but prevalence in SLE is only 30%)
What drug should MOST patients with SLE be on whether on immunosuppressive agents or not as it controls RASH and ARTHRITIS, prevents FLARES, increases long-term SURVIVAL and reduces ORGAN DAMAGE, THROMBOSIS and BONE LOSS?
Hydroxychloroquine (an anti-malarial drug)
What medications are used for SLE when mild rash and joint symptoms occur?
Low-dose corticosteroids and NSAID’s
What medication is used for SEVERE SLE FLARES?
High-Dose CORTICOSTEROIDS
When are immunosuppressive agents such as Methotrexate, Azathioprine, Mycophenolate Mofetil and Cyclophosphamide used to treat SLE?
When SLE flares require long-term high dose corticosteroids and steroid-sparing agents are needed
What is the PREFERRED immunosuppressive agent used for LUPUS NEPHRITIS (Proliferative Nephritis) though it causes hemorrhagic cystitis and urinary bladder cancer?
Cyclophosphamide
Which is the ONLY anti-TNF-α inhibitor used for SLE?
Belimumab
What vaccination is HIGHLY recommended for women with SLE due to the HIGHT risk of cervical dysplasia in this disease?
HPV vaccine
What SLE therapy REUCES FERTILITY?
Cyclophosphamide
What are the ONLY three (3) medications that CAN be used in PREGNANT patients with SLE?
Hydroxychloroquine, Prednisone and Azathioprine
What are patients with SLE and antiphospholipid Ab’s with h/o recurrent EARLY pregnancy LOSS or SINGLE LATE pregnancy loss treated with DURING pregnancy?
ASPIRIN + HEPARIN
Neonatal Lupus Erythematosus is caused by transplacental passage of maternal autoantibodies to the fetus and is found to be of much higher prevalence when what lab tests are positive in the mothers?
Anti-Ro (SSA) and anti-La (SSB) Ab’s (subacute cutaneous LUPUS markers as well)
In women who have SLE and are antiphospholipid Ab POSITIVE, since Combination Oral Contraceptive Pills are CONTRAINDICATED, what contraceptive methods CAN be used?
Barrier method (condom, diaphragm, sponge, etc. OR PROGESTERONE-ONLY methods)
What systemic inflammatory disease increases the risk of PREECLAMPSIA in pregnant women?
SLE
VASCULOPATHY affecting WOMEN in their 30’s and 40’s with FIBROSIS of the DERMIS and visceral ORGANS is known as?
Systemic Sclerosis (Scleroderma)
Systemic Sclerosis that LIMITS its SKIN involvement to the FACE and DISTAL EXTREMITIES is called what?
Limited Cutaneous Systemic Sclerosis
A subset of Limited Cutaneous Systemic Sclerosis that presents with Calcinosis, Raynaud, Esophageal Dysmotility, Sclerodactyly and Telangiectasias is called what? what LUNG disease are these patients at higher risk of developing?
CREST Syndrome; Pulmonary ARTERY HTN
Systemic Sclerosis with DIFFUSE SKIN involvement (not just face and distal extremities) is associated with what LUNG and KIDNEY diseases?
Interstitial Lung Disease and Scleroderma Renal Crisis
Tightness, Thickening and Swelling of the digits (sclerodactyly) with pitting or ulceration of the ends of the fingers and Raynaud phenomenon are seen in?
Systemic Sclerosis (Scleroderma)
POSITIVE ANA (95%), Anti-CENTROMERE Ab, Anti-Scl-70 Ab, Anti-U3-RNP Ab, Anti-PM-Scl Ab and Anti-Th/To Ab?
Systemic Sclerosis (Scleroderma)
Which Ab’s are associated with Systemic Sclerosis with Pulmonary Artery HTN?
Anti-CENTROMERE, Anti-U3-RNP and Anti-Th/To Ab’s
Why should SYSTEMIC CORTICOSTEROIDS be AVOIDED in the treatment of Systemic Sclerosis (Scleroderma)?
Because of the HIGH-RISK of developing Scleroderma Renal CRISIS
What is PRURITUS treated with in Systemic Sclerosis (Scleroderma)?
Antihistamines and Emollients
What are the EARLY symptoms of Digital Swelling and Dermal EDEMA treated with in Systemic Sclerosis (Scleroderma)?
Methotrexate or Imatinib
The inflammatory, non-erosive arthritis that can develop in SYSTEMIC SCLEROSIS (Scleroderma) due to involvement of the skin with loss of JOINT mobility and tendon friction rubs is BEST treated with what?
Methotrexate
How are patients with inflammatory MYOPATHY with proximal muscle weakness and elevated CK and aldolase in the setting of SYSTEMIC SCLEROSIS (scleroderma) treated?
CAUTIOUSLY with corticosteroids (can cause Scleroderma Renal Crisis)
Pt who has Raynaud phenomenon and develops pitting of the end of their digits or ulcers/gangrene mot likely is developing what?
Systemic Sclerosis (Scleroderma)
Raynaud phenomenon, Digital pitting/ulcers/gangrene, Nail changes (abnormal nail fold capillaries), lung, myocardial, GI tract and Kidney disease seen in SYSTEMIC SCLEROSIS are caused by what process?
Intimal proliferation and resulting luminal obliteration of terminal arterioles and capillaries (vascular disease)
How is Raynaud phenomenon treated besides avoidance of cold temperatures?
Ca-channel blockers, Antiplatelet agents (aspirin) and Topical nitrates
What is done for patients with SEVERE Raynaud phenomenon in danger of developing gangrene and digit loss?
Treatment with SILDENAFIL, Prostacyclin Analogues (iloprost), Pain management (to avoid sympathetic-mediated vasospasm) and possible regional sympathetic blockade vs sympathectomy
What is the most COMMON GI manifestation of SYSTEMIC SCLEROSIS (Scleroderma) as it precedes most other manifestations of the disease?
Esophageal Dysmotility with reflux and dysphagia
Why can you see GI bleeding with anemia and iron deficiency in a patient with Systemic Sclerosis (Scleroderma)?
Because of TELANGIECTASIAS
Besides Esophageal Dysmotility (crEst), GI Bleed from Telangiectasias (cresT) and wide-mouth diverticula, what other GI disease can be associated with Systemic Sclerosis (Scleroderma)?
Primary Biliary Cirrhosis (PBC)
What causes ACUTE onset of HTN, AZOTEMIA (BUN, Cr) and Microangiopathic HEMOLYTIC ANEMIA?
Scleroderma Renal Crisis (can be triggered by use of systemic corticosteroids in the treatment of Systemic Sclerosis (Scleroderma)
What should a patient that presents with elevated blood pressure and Cr levels with SYSTEMIC SCLEROSIS (Scleroderma) be treated with?
ACE-I’s (to protect kidneys in Scleroderma Renal Crisis) EVEN in the setting of kidney failure and dialysis
What are the two manifestations of LUNG disease in SYSTEMIC SCLEROSIS (Scleroderma)?
Interstitial Lung Disease and Pulmonary Artery HTN
Patients with Systemic Sclerosis (Scleroderma) and what LAB value have the HIGHEST risk of developing Interstitial Lung Disease (ILD)?
Anti-Scl-70 Ab’s
Reticular linear opacities, Ground Glass Opacities on CT of the chest with decreased Pulmonary Function Tests, DLCO and LUNG volumes in a patient with SYSTEMIC SCLEROSIS (Scleroderma) suggests what disease?
Interstitial Lung Disease (ILD)
How is Interstitial Lung Disease treated in a patient with Systemic Sclerosis (Scleroderma)?
Cyclophosphamide with LOW dose steroids x 1 year FOLLOWED by Azathioprine
Dyspnea and Exercise Intolerance in a patient with Systemic Sclerosis (Scleroderma) with an INCREASED PULMONIC component of S2 and a PERSISTENTLY-SPLIT S2 suggests what disease?
Pulmonary Artery HTN
ECHO that demonstrates RV dilation or RIGHT sided regurgitant valve lesions is suggestive of what condition seen in patients with Systemic Sclerosis (Scleroderma)?
Pulmonary Artery HTN
How is Pulmonary Artery HTN BEST measured PRIOR to starting treatment?
RIGHT heart catheterization
How is Pulmonary Artery HTN treated in patients with Systemic Sclerosis (Scleroderma)?
Oxygen supplementation, Anticoagulation as well as Sildenafil, prostacyclin analogues (iloprost) and endothelin receptor antagonists (bosentan, ambrisentan)
What cardiac manifestations are seen in patients with Systemic Sclerosis (Scleroderma)?
Pericardial Effusions (usually asymptomatic), Myocardial Fibrosis and Contraction Band Necrosis
A localized form of Scleroderma (Systemic Sclerosis) presenting with indurated plaques on the torso and proximal extremities WITHOUT visceral manifestations OR Raynaud phenomenon is called?
MORPHEA
A woody-induration of the extremities SPARING the hands and face with a peripheral EOSINOPHILIA with a skin biopsy showing LYMPHOCYTES, PLASMA CELLS and EOSINOPHILS infiltrating the deep fascia is a disorder associated with Scleroderma (Systemic Sclerosis) that is called?
Eosinophilic Fasciitis
Long-standing insulin-dependent DM complication presenting with noninflammatory, INDURATED plaques over the shoulder girdle, neck and upper extremities is called?
SCLEREDEMA
Skin biopsy demonstrating deposition of MUCIN with a large number of Stellate Fibroblasts in the dermis of the FACE, TORSO and UPPER Extremities occurring in the setting of Multiple Myeloma and Amyloidosis is called?
Scleromyxedema
What Systemic Sclerosis (Scleroderma) manifestation poses the GREATEST RISK to pregnancy?
Scleroderma Renal Crisis
Although HIGHLY TERATOGENIC, what medication MUST be used in PREGNANT patients who develop Scleroderma Renal Crisis in order to preserve Kidney function?
ACE-I’s
What are the two expected EFFECTS of Systemic Sclerosis (Scleroderma) in PREGNANT patients?
Premature Birth and LOW BIRTH WEIGHT
An autoimmune syndrome that targets exocrine glands and presents with keratoconjunctivitis sicca (dry eyes - gritty and blurry - corneal ulcers) and xerostomia (dry mouth - dysphagia, dental caries and candidiasis) with PAROTID GLAND Enlargement?
Sjögren Syndrome
What two autoimmune diseases have manifestations of Sjögren Syndrome?
RA and SLE
What malignancy is seen most COMMONLY in patients with Sjögren Syndrome?
Lymphadenopathy and B-cell MALT-LYMPHOMA
Positive ANA, RF, anti-Ro (SSA) and anti-La (SSB) Ab’s, elevated IgG (hypergammaglobulinemia) and Cryoglobulins with abnormal Lacrimal and Parotid Gland Function?
Sjögren Syndrome
In a patient with Sjögren Syndrome and progressive UNILATERAL PAROTID Gland enlargement, what should be done and why?
Parotid Gland BIOPSY - to assess for LYMPHOMA
How are Dry Eyes and Dry Mouth treated in Sjögren Syndrome?
Symptomatically: artificial tears, punctal occlusion (of tear-drainage duct), sugar-free lozenges, lubricating agents and muscarinic agonists (pilocarpine, cevimeline)
How are more SEVERE extraglandular manifestations of Sjögren Syndrome (arthralgia, etc.) treated?
NSAIDS, Hydroxychloroquine, corticosteroids and immunosuppressive drugs as needed
If a patient with Sjögren Syndrome develops Palpable Purpura, Low C4 levels, Mixed Monoclonal Cryoglobulinemia and/or PERSISTENT PAROTID gland enlargement, what should be suspected?
LYMPHOMA
What is seen on a BIOPSY of the LIP (minor salivary gland) in a patient with Sjögren Syndrome?
Focal LYMPHOCYTIC infiltration
Overlapping CLINICAL features of SLE, Polymyositis and Systemic Sclerosis (Scleroderma) mostly seen in WOMEN aged 20-30 with POSITIVE anti-U1-RNP Ab’s is what disease?
Mixed Connective Tissue Disease (MCTD)
What CLINICAL feature that is NOT seen in SLE, Polymyositis or Systemic Sclerosis (Scleroderma) is seen in 25% of patients with Mixed Connective Tissue Disease (MCTD) - positive anti-U1-RNP Ab’s?
Trigeminal Neuropathy
ANA (high-titer SPECKLED pattern) + anti-U1-RNP (high-titer)?
Mixed Connective Tissue Disease (MCTD)
What is the HIGHEST RISK for DEATH in patients with Mixed Connective Tissue Disease (MCTD) which is the same for patients with Systemic Sclerosis (Scleroderma)?
Pulmonary Artery HTN (requires RIGHT heart CATH to measure and diagnose)
How is Pulmonary Artery HTN BEST treated if identified EARLY?
Aggressive CYCLOPHOSPHAMIDE therapy (risk of hemorrhagic cystitis and bladder cancer)
What is the MOST common INFLAMMATORY arthritis in the US?
GOUT
Kidney disease, HTN, Obesity, DM, Cardiovascular disease and elevated SERUM URATE levels can cause what arthritis?
GOUT
Organ Meats, Seafood, Obesity, Increased Cell Turnover result in elevated URIC Acid due to increased breakdown of PURINES causes what arthritis?
GOUT
Leukemia, Lymphoma, Hemolytic Anemia, Polycythemia Vera, Ineffective Hematopoiesis, Psoriasis, Chemotherapy-Induced Tumor Lysis (leukemia, lymphoma) and treatments with Growth Factors (G-M-CSF, G-CSF, Erythropoietin) all can cause what arthritis?
GOUT
Dehydration and Alkalosis (provoke SODIUM or Proton retention), acids such as LACTIC acid (sepsis) and KETO acid (DM) as well as LEAD toxicity, inhibit renal transporters and block URATE excretion resulting in what arthritis?
GOUT
Diets high in FRUCTOSE (fruit, sugar substitutes) as well as Organ Meats, Seafood and Alcohol (beer) result in HIGH PURINE consumption that can cause what arthritis?
GOUT
THIAZIDE and LOOP Diuretics, NICOTINIC Acid (Niacin “vitamin B3”), INH and PYRAZINAMIDE block URATE Excretion causing HYPERuricemia and therefore what arthritis?
GOUT
What is the difference in JOINT involvement of GOUT in acute vs chronic disease?
ACUTE GOUT - usually involves the big toe
CHRONIC GOUT - polyarticular
RED, HOT, TENDER Joint with a low-grade fever that resolves without treatment over DAYS to WEEKS, if acute, is usually seen at the FIRST metatarsal-phalangeal Joint of the foot (big toe) and if chronic, can be any joint or multiple joints?
GOUT
Urate crystal deposition surrounded by local inflammation in subcutaneous sites or cartilage or SUB-chondral bone causing erosive damage and can be infected?
Tophi (gout)
ACUTE MONOarticular or POLYarticular INFLAMMATORY arthritis in MEN (rarely in post-menopausal women) with fever (low grade), is likely what? Should ALWAYS be evaluated for what? Why?
GOUT
R/O Septic Joint
Rheumatologic EMERGENCY
EXCEPT for PODAGRA (acute GOUTY inflammation of the first metatarsal-phalangeal joint of the foot) what is required for ALL other acutely-inflamed joints?
ASPIRATION to rule out septic joint
Synovial fluid with a HIGH neutrophil count (>3,000/µL) or needle-shaped, negatively birefringent crystals under polarized microscopy indicates a diagnosis of what?
GOUT
If a septic JOINT is suspected, what should be done after aspirating the joint?
Gram stain and culture and empiric antibiotic coverage
PUNCHED-OUT lesions in subchondral bone indicates what?
CHRONIC GOUT (urate deposition or massive, joint-destroying tophi)
How is an ACUTE GOUT attack treated?
NSAID’s (indomethacin or others), COLCHICINE (fast-acting), Corticosteroids (intra-articular work best)
What should NOT be done in an ACUTE GOUT attack?
DO NOT START or STOP urate-lowering drugs (can precipitate further attacks)
What should be done BEFORE intra-articular injection of corticosteroids in a patient with an ACUTE gout attack?
RULE OUT Joint infection
Is Calcium Phosphate Deposition visible on polarizing microscopy?
NO
What patients with GOUT require PROPHYLAXIS?
Those with GOUT attacks ≥2/year OR if they have TOPHI
What are the two (2) BEST drug choices for GOUT prophylaxis in patients with GOUT of any cause (primary underexcreters and secondary (due to kidney disease) underexcreters?
Allopurinol and Febuxostat (both block urate production via xanthine oxidase (purine salvage enzyme) inhibition
What is the BEST drug choice for the treatment of GOUT in PRIMARY UNDEREXCRETERS (no kidney disease)?
Probenecid (increases URATE excretion by blocking URAT-1 transporter) however can cause kidney STONES
How can you tell whether a patient with gout is a PRIMARY underexcreter or not?
24-hour URINE collection (measure for volume - to evaluate for normal kidney function - and for uric acid content to evaluate for underexcretion)
What should a patient be started on IN CONJUNCTION with ANY urate-lowering therapy for at LEAST 6 MONTHS?
Colchicine (in order to prevent acute flares of GOUT seen with ANY mobilization of urate)
What should be done with all urate-lowering therapies?
Tapered to the lowest effective dose (achieve a serum urate level
What dietary and medication modifications should be made in all GOUT patients?
Decrease purine (meats and fructose) intake, decrease alcohol intake and begin weight loss, substitute loop and thiazide diuretics if possible
How are GOUTY Tophi treated?
By lowering serum urate to
What is the ONLY thing that should be addressed in an ACUTE GOUT attack?
INFLAMMATION (do NOT attempt to raise or lower serum urate levels)
What type of crystals are deposited and in turn cause inflammation in pseudoGOUT?
Calcium Pyrophosphate (positively birefringent, rhomboid-shaped crystals)
Where do the Calcium Pyrophosphate crystals usually deposit?
Cartilage (chondrocalcinosis - seen on x-rays of the knees or wrists) or JOINTs
What are the commonly affected JOINTS by Calcium Pyrophosphate Deposition Disease (that allows distinction from Osteoarthritis with chondrocalcinosis)?
WRIST and the Second and Third metacarpophalangeal joints (hand)
Hypercalcemia, hyperphosphatemia, elevated ESR, CRP in the setting of an ACUTE inflammatory monoarthritis and x-rays with chondrocalcinosis?
pseudoGOUT (Calcium Pyrophosphate Deposition Disease - CPPD)
What makes a definitive diagnosis of pseudoGOUT (Calcium Pyrophosphate Deposition Disease - CPPD)?
Joint aspiration demonstrating POSITIVELY-birefringent RHOMBOID-shaped crystals and neutrophils and crystals within the neutrophils)
How is pseudoGOUT (Calcium Pyrophosphate Deposition Disease - CPPD) treated?
With control of INFLAMMATION in the SAME way GOUT is (NSAID’s - indomethacin, intra-articular corticosteroid injections and Colchicine)
In contrast to GOUT, how are FREQUENT pseudoGOUT attacks prophylactically treated?
With LOW-dose Colchicine or NSAID’s
A disease of crystal deposition in which the crystals are made up of HYDROXYAPATITE, are SMALL and NON-BIREFRINGENT, occur in patients ≥50 and in joints affected by osteoarthritis and can be HIGHLY DESTRUCTIVE?
BASIC CALCIUM PHOSPHATE (BCP) deposition disease
How is BASIC CALCIUM PHOSPHATE (BCP) deposition disease treated?
Same as pseudoGOUT with NSAID’s (indomethacin), intra-articular injection of corticosteroids and Colchicine
Why must SEPTIC joints be diagnosed and treated EMERGENTLY?
Because BACTERIAL joint infections can damage the joints in DAYS to WEEKS (unlike fungal) - viral infections do not damage joints
A joint that presents with WARMTH, REDNESS, SWELLING and PAIN is likely?
An INFECTED JOIN and infection MUST be ruled out
What is the CAUSE of MOST joint infections?
Hematogenous spread (pneumonia, UTI, Endocarditis, Abscess), direct-injury spread is also common
What patients are MORE SUSCEPTIBLE to JOINT infections?
Those with established JOINT DISEASE (chronic gout, osteoarthritis)
What are ESSENTIAL LABS when evaluating a patient with a possible JOINT infection?
JOINT ASPIRATION (fluid analysis, leukocyte count, gram stain and culture) PRIOR to initiating ANTIBIOTICS, CBC (leukocytosis), ESR, CRP, BLOOD CULTURES (even WITHOUT FEVER), URINALYSIS
What is considered a positive BACTERIAL joint infection?
Neutrophilic Leukocytosis ≥50,000/µL
What is the most COMMON organism involved in JOINT infections?
Gram POSITIVE, Staph .aureus
What is the most COMMON organism involved in PROSTHETIC JOINT infections?
Gram POSITIVE Staph. epidermidis
What is the most COMMON organism involved in JOINT infections in DIABETICS?
Gram POSITIVE Streptococcus
These types of organisms, affect LARGE JOINTS (KNEE) and usually MONOARTICULAR?
Gram POSITIVE (staph. aureus, staph. epidermidis, strep)
How can you differentiate between a bacterial vs a fungal/viral JOINT infection by history alone?
Bacterial infections occur over DAYS to WEEKS (rapid)
A SINGLE JOINT infection (KNEE, WRIST or ANKLE) in a young, sexually active adult, with FEVER, CHILLS and tenosynovitis (swollen hands or feet) and a vesicopustular RASH?
Gonorrhea (Gram NEGATIVE)
What special CULTURE MEDIA is REQUIRED for diagnosing Neisseria Gonorrhea?
Thayer-Martin
Do NEGATIVE joint fluid studies rue out Gonorrhea?
NO
If suspicion for Gonorrheal JOINT infection is HIGH but JOINT fluid studies are negative, what other studies should be done?
Direct culture or PCR of specimens from URETHRA, CERVIX, URINE, THROAT and RECTUM
What type of bacteria cause JOINT infections in DIABETICS, IMMUNOCOMPROMISED patients and elderly patients ≥65?
Gram NEGATIVE bacteria
Patients with Sickle Cell Anemia are susceptible to what particular Gram NEGATIVE organism that can also cause JOINT infections?
Salmonella
Injection DRUG users (IVDA) are susceptible to what particular Gram NEGATIVE organism that can also cause JOINT infections?
Pseudomonas
What are the most common Gram NEGATIVE bacteria to cause JOINT infections?
P.aeruginosa and E.coli (fever, sepsis)
Chronic MONOARTICULAR arthritis with large effusion usually affecting the KNEE, HIP or ANKLE, confirmed by PCR and can occur MONTHS to YEARS after the infection?
LYME arthritis
Monoarticular arthritis with HIGH ESR and CRP, caused by an infection that can occur in an INTERVERTEBRAL DISK with DESTRUCTIVE OSTEOMYELITIS involving adjacent VERTEBRA requiring vertebral biopsy?
POTT Disease (mycobacterium tuberculosis) - can occur WITHOUT lung involvement
An organism present in freshwater and saltwater causing infection after SKIN PUNCTURE from contaminated objects (fish hooks) or by contacting FISH or WATER with breaks in the SKIN - aquarium fish with infection LOCALIZED to puncture area involving the SKIN, TENDONS and adjacent JOINTS?
Mycobacterium MARINUM
What skin manifestation can occur with infection with Mycobacterium Marinum?
Nodular papules that ulcerate
What patients are susceptible to JOINT infections with fungi (candida, aspergillus, cryptococcus, coccidioides, blastomyces, sporothrix, histoplasma)?
Immunocompromised
SCHOOLWORKER or PARENT presents in the WINTER or SPRING with a flu-like illness with FEVER and POLYARTHRITIS after exposure to a child with a facial rash and symptoms resolve after several weeks with lingering arthralgias and myalgias for months to years?
Parvovirus-B19
How is Parvovirus-B19 diagnosed?
IgM anti-parvovirus Ab’s
Single-stranded RNA virus that presents with COUGH, FEVER, LYMPHADENOPATHY and a MORBILLIFORM RASH with polyarthralgia?
Rubella
Erosion or loosening around a prosthetic joint implantation site with elevated leukocyte counts, ESR and CRP with swelling, inflammation or pain?
Prosthetic Joint Infection
When is a PROSTHETIC JOINT infected if symptoms occur ≤3 months? >3 months?
≤3 months - During implantation
>3 months - After implantation (hematogenous)
What is MANDATORY for a suspected PROSTHETIC JOINT infection?
Orthopedic Consult (may need open debridement or removal)
How are INFECTED JOINTS treated?
CULTURE FIRST, then EMPIRIC antibiotic treatment until Gram stain and Culture results focus therapy
What are the ANTIBIOTICS used to treat a suspected Gram POSITIVE JOINT infection? what if suspecting MRSA?
OXACILLIN/NAFCILLIN or CEFAZOLIN
If suspecting MRSA - VANCOMYCIN or LINEZOLID
What is the ANTIBIOTIC used to treat a suspected GONORRHEAL JOINT INFECTION?
CEFTRIAXONE
What are the ANTIBIOTICS used to treat a suspected Gram NEGATIVE Enteric (e.coli) JOINT infection? P.aeruginosa?
Enteric - CEFTRIAXONE or CEFOTAXIME
P.aeruginosa - CEFTAZIDIME + gentamycin
How long are JOINT infections treated for and how?
2-4 WEEKS, starting with IV Abx
How is a Gonorrheal JOINT infection treated?
Start with IV CEFTRIAXONE then change to PO CIPROFLOXACIN and continue for 7-14 DAYS
ALL Gonorrheal infections, whether involving JOINTS or not should be treated how?
With BOTH CEFTRIAXONE (for gonorrhea) and DOXYCYCLINE or AZITHROMYCIN for CHLAMYDIAL co-infection
How is a tubercular JOINT infection treated (POTT disease, etc.)?
With 3-4 DRUGS for ≥6 MONTHS (INH, Rifampin, Pyrazinamide, Ethambutol or Streptomycin)
How is a Mycobacterium MARINUM Joint infection treated?
Minocycline or Clarithromycin or TMP-SMX
For VIRAL JOINT infections, besides those caused by Hep B or Hep C which require antiviral-therapy, how are the rest treated?
Symptomatically with NSAID’s as these are self-limited
How should the INFECTED JOINT be treated besides ANTIBIOTIC therapy?
With REPEATED DAILY joint fluid ASPIRATIONS until fluid STOPS ACCUMULATING
When is SURGICAL DRAINAGE/DEBRIDEMENT of an INFECTED JOINT needed?
When the joint is NOT ACCESSIBLE to perform needle drainage, when there are LOCULATIONS and when the JOINT SPACE is IRREGULAR
How is an INFECTED PROSTHETIC JOINT treated?
Joint is REMOVED, an ANTIBIOTIC SPACER is placed for WEEKS to MONTHS and then a NEW one is placed
What do MOST patients feel in the RECOVERY phase of a treated FUNGALLY or BACTERIALLY INFECTED JOINT?
STIFFNESS
If a patient with an INFECTED PROSTHETIC JOINT is NOT a candidate for the removal/spacer/replacement treatment protocol, what can be done?
DEBRIDEMENT of the JOINT with CHRONIC antibiotic suppression
What are the three (3) IDIOPATHIC INFLAMMATORY MYOPATHIES in which muscle weakness is caused by inflammation?
Polymyositis, Dermatomyositis, Inclusion-body myositis
What is characteristically seen in MUSCLE biopsies of patients with an INFLAMMATORY MYOPATHY?
LYMPHOCYTIC infiltrates with evidence of NECROSIS and REGENERATION
A myositis that has a BIMODAL distribution, appearing
Dermatomyositis
ACUTE, SYMMETRICAL, PROGRESSIVE, PAINLESS (or little pain) PROXIMAL muscle weakness involving NECK flexor muscles and RESPIRATORY muscles in LATE disease?
Dermatomyositis and Polymyositis
SLOW to progress, INSIDIOUS in BOTH PROXIMAL and DISTAL MUSCLE groups (quadriceps, wrists, fingers, swallowing), USUALLY symmetric and affects MEN ≥50?
INCLUSION-BODY MYOSITIS
MYOSITIS that presents with PHOTOSENSITIVE rashes over the FACE (heliotrope rash), CHEST (V-sign, Shawl sign), HANDS, ELBOWS and KNEES (Gottron papules over the extensor joints) with Nail-Fold capillary abnormalities and cuticular hypertropy?
DERMATOMYOSITIS
Roughened, Erythematous, Hyperkeratotic Fissuring of the PALMAR and LATERAL aspects of the FINGERS (MECHANIC’s HANDS) is found in what diseases?
Dermatomyositis and Polymyositis
What is AMYOPATHIC Dermatomyositis?
Dermatomyositis WITHOUT muscle involvement
What are the EXTRAMUSCULAR manifestations that DERMATOMYOSITIS and POLYMYOSITIS have that INCLUSION-BODY MYOSITIS does not?
Fever, Fatigue, Weight-loss, Raynaud phenomenon, Arthralgia, Arthritis, Pulmonary, Cardiac and GI symptoms
What are the PULMONARY manifestations of Dermatomyositis and Polymyositis?
Interstitial Lung Disease (ILD) causing: hypoventilation (due to respiratory muscle weakness), aspiration pneumonia, Pulmonary Artery HTN
How is Interstitial Lung Disease diagnosed in patients with Dermatomyositis and Polymyositis?
CT chest and Pulmonary Function Testing
The presence of AMINOACYL-TRANSFER RNA SYNTHETASE (anti-Jo-1) Ab’s in patients with Dermatomyositis or Polymyositis is STRONGLY suggestive of what?
Presence of Interstitial Lung Disease (ILD) - pneumonitis, pneumonia, bronchiolitis obliterans with organizing pneumonia
Dysphagia with weakness of the swallowing muscles occurs LATE in the course of which myositis?
Inclusion-Body Myositis
What labs are generally elevated in a patient with MYOSITIS and fluctuate with disease activity?
CK, Aldolase, ALT, AST
The presence of the anti-Mi-2 Ab is a good prognostic feature and associated with what manifestation of what disease?
Dermatomyositis with classic RASH, mild myositis
What are imaging modalities with US or MRI (in obese patients) used for in MYOSITIS?
To identify optimal biopsy sites
Short-Duration, Small, Low-Amplitude, Polyphasic Potentials, Fibrillation Potentials at Rest and Bizarre, High-Frequency, Repetitive Discharges are EMG findings of what condition?
Myositis
If performing an EMG in a patient with Myositis, where should a biopsy be obtained from relative to the EMG?
The opposite (contralateral) side, away from where the EMG was done
What is found SPECIFICALLY on immunostaining of biopsies in DERMATOMYOSITIS?
B-cells and CD4-positive T-cells (vascular endothelium is under attack)
What is found SPECIFICALLY on immunostaining of biopsies in Polymyositis and IBM?
CD8-positive T-cells (myofibril is under attack)
What is found in Dermatomyositis on skin (not muscle) biopsy?
Interface dermatitis
What is the proper BIOPSY TECHNIQUE for MYOSITIS?
Surgical, NOT needle
How are Dermatomyositis and Polymyositis treated?
HIGH-dose corticosteroids until CK levels normalize, then taper steroids
After steroid treatment of a MYOSITIS, a patient has PROXIMAL muscle weakness however normal CK levels, why?
They developed steroid-induced myopathy
What is used for the treatment of Steroid-Sparing, steroid-resistant MYOSITIS or myositis with Interstitial Lung Disease (ILD)?
Immunosuppressive therapy such as Methotrexate, Azathioprine or IVIgG
How is the RASH of Dermatomyositis treated?
Hydroxychloroquine or TOPICAL steroids or TACROLIMUS with SUNSCREEN
What underlying disease is strongly-associated with Dermatomyositis, Polymyositis and Inclusion-Body Myositis?
Malignancy (OVARIAN cancer and Adenocarcinomas)
How do you screen a patient with Idiopathic Inflammatory Myopathy (dermatomyositis, polymyositis and inclusion-body myositis) for malignancy?
Age-appropriate testing (urinalysis, CXR, colonoscopy, PSA, Pap-smear with CA-125 and pelvic US
What are the most common causes of death in patients with idiopathic inflammatory myopathies (dermatomyositis, polymyositis and inclusion-body myositis)?
Malignancies and Infections (LUNG)
What is the MOST important predictor of mortality in a patient with idiopathic inflammatory myopathy (dermatomyositis, polymyositis and inclusion-body myositis)?
Age
Giant-Cell Arteritis (temporal arteritis), Polymyalgia Rheumatica and Takayasu Arteritis are what category of VASCULITIS?
Large-Vessel Vasculitis
This Large-Vessel Vasculitis affects the EXTRACRANIAL arteries of the head and neck, the THORACIC AORTA and its major branches?
Giant-Cell Arteritis (Temporal Arteritis)
Biopsy of an affected vessel with this vasculitis shows a TRANSMURAL LYMPHOCYTIC INFILTRATE with DISRUPTION of the INTERNAL ELASTIC LAMINA?
Giant-Cell Arteritis (Temporal Arteritis)
This Large-Vessel Vasculitis commonly affects WOMEN ≥50 and MULTINUCLEATED GIANT CELLS are found within the VESSEL WALL or ADVENTITIA?
Giant-Cell Arteritis (Temporal Arteritis)
New-onset of TEMPORAL or OCCIPITAL HA in a woman ≥50 with FEVER and FATIGUE, STIFFNESS and PAIN in the HIP and SHOULDER, JAW Claudication and SCALP TENDERNESS, Aortic Valve Regurgitation and potential VISUAL loss from involvement of the OPHTHALMIC artery?
Giant-Cell Arteritis (Temporal Arteritis)
Audible BRUITS over the neck or subclavian fossa, VERY HIGH ESR and CRP levels, tenderness over the TEMPORAL Arteries with ANEMIA and THROMBOCYTOSIS?
Giant-Cell Arteritis (Temporal Arteritis)
FUSIFORM luminal narrowing of the CAROTID, INNOMINATE or SUBCLAVIAN arteries is characteristic of what Large-Vessel VASCULITIS?
Giant-Cell Arteritis (Temporal Arteritis)
How is Giant-Cell Arteritis (Temporal Arteritis) treated?
CORTICOSTEROIDS - WITHOUT awaiting biopsy results and LOW-DOSE Aspirin
What can be done if a patient with Giant-Cell Arteritis (Temporal Arteritis) develops multiple flares when steroids are tapered?
Can try Methotrexate, Azathioprine or Mycophenolate Mofetil
The classic TRIAD of EMBOLISM, INTRACARDIAC OBSTRUCTION leading to pulmonary congestion or CHF and symptoms of FATIGUE, WEIGHT LOSS and FEVER?
Atrial MYXOMA
Pt with SEVERE atherosclerosis presents after ABDOMINAL TRAUMA or AORTIC SURGERY or ANGIOGRAPHY but can also occur with HEPARIN, WARFARIN or THROMBOLYTIC THERAPY with LIVEDO RETICULARIS and LOCALIZED SKIN NECROSIS?
Multiple CHOLESTEROL EMBOLI
A Large-Vessel VASCULITIS that is CLOSELY ASSOCIATED with Giant-Cell Arteritis (Temporal Arteritis) and presents with PAIN and STIFFNESS in the PROXIMAL LIMBS associated with ELEVATED ESR and CRP with LIMITATION in the RANGE of MOTION of the SHOULDER, NECK and HIP with FATIGUE and FEVER?
Polymyalgia Rheumatica
How is POLYMYALGIA RHEUMATICA treated?
With a PROLONGED (6 MONTH) course of LOW-DOSE Corticosteroids
What can be used as a STEROID-SPARING agent in POLYMYALGIA RHEUMATICA?
METHOTREXATE
Large-Vessel VASCULITIS that affects the AORTA, its MAJOR BRANCHES and the PULMONARY ARTERIES with BIOPSIES demonstrating LYMPHOPLASMACYTIC and GRANULOMATOUS INFILTRATE through the vessel wall (TRANSMURAL), usually seen in WOMEN in their 20’s-40’s?
TAKAYASU ARTERITIS
MONTHS of low-grade FEVER, FATIGUE, MALAISE, MYALGIA and ARTHRALGIA, WEIGHT LOSS and SIGNIFICANTLY-ELEVATED ESR and CRP with BRUITS heard over the CAROTID, SUBCLAVIAN, RENAL or ILIAC vessels with UPPER or LOWER EXTREMITY CLAUDICATION?
TAKAYASU ARTERITIS
HTN due to Renal Artery Stenosis with DIFFERENTIAL SYSTOLIC blood pressures and PULSE deficits in the extremities?
TAKAYASU ARTERITIS
How is TAKAYASU ARTERITIS Diagnosed?
Angiography, CT-angiography or MR-angiography demonstrating characteristic NARROWING of the AORTA and its MAJOR BRANCHES
How is the ACUTE (inflammatory) phase of TAKAYASU Arteritis treated?
HIGH-DOSE Corticosteroids
If TAKAYASU Arteritis is steroid-refractory or disease is SEVERE, what else can be used daily?
Cyclophosphamide (risk of hemorrhagic cystitis and bladder cancer)
What steroid-sparing drugs can be used to treat TAKAYASU Arteritis?
Methotrexate or anti-TNF-α inhibitors
What prophylactic drugs should be used in ANY patient with TAKAYASU Arteritis to prevent premature atherosclerosis and thrombosis?
Aspirin and Statins
POLYARTERITIS NODOSA and KAWASAKI DISEASE are what caliber VASCULITIS?
MEDIUM-VESSEL Vasculitis
What does a BIOPSY of a patient with POLYARTERITIS NODOSA demonstrate?
Neutrophils, Mononuclear cells that invade the artery walls and NECROTIZING arteritis with medium-artery aneurysms
This EDIUM-VESSEL Vasculitis is often found in older patients (40-60) with Hep B, with fever, arthralgia, myalgia, abdominal pain and weight loss with PERIPHERAL NERVE manifestations such as MONONEURITIS and renovascular HTN?
POLYARTERITIS NODOSA
MONONEURITIS, TESTICULAR PAIN, painful CUTANEOUS NODULES, SKIN ULCERS, PALPABLE PURPURA, LIVEDO RETICULARIS with ELEVATED ESR, ANEMIA, THROMBOCYTOSIS and LEUKOCYTOSIS associated with Hep B?
POLYARTERITIS NODOSA
Why should the KIDNEYS NOT BE BIOPSIED in patients with POLYARTERITIS NODOSA?
Because of potential hemorrhage from medium-vessel aneurysms
What are the BEST imaging studies for patients with suspected POLYARTERITIS NODOSA?
Angiography or CT-angiography
How is POLYARTERITIS NODOSA treated?
HIGH-DOSE Corticosteroids with slow taper
How is steroid-resistant POLYARTERITIS NODOSA or SEVERE disease treated?
Cyclophosphamide (risk of hemorrhagic cystitis and bladder cancer)
How is POLYARTERITIS NODOSA BEST treated in the setting of Hep B?
With Corticosteroids AND anti-viral therapy (ENTECAVIR)
Mainly a MEDIUM-VESSEL Vasculitis that occurs in CHILDREN but also seen in ADULTS, especially with HIV?
KAWASAKI disease
This MEDIUM-VESSEL Vasculitis presents with FEVER, CERVICAL Lymphadenopathy, CONJUNCTIVITIS, ORAL MUCOSITIS (TONGUE), MULTIPLE-Joint arthritis and a RASH (back and PROXIMAL UE & LE)?
KAWASAKI disease
MEDIUM-VESSEL Vasculitis with STERILE PYURIA besides the usual VASCULITIS lab findings (anemia, thrombocytosis, leukocytosis)
KAWASAKI disease
This MEDIUM-VESSEL Vasculitis can cause Acute Coronary Syndrome or PERIPHERAL Vascular Occlusion and Coronary Artery Aneurysms in children?
KAWASAKI disease
Unique from the treatment of other VASCULITIS, this disease is treated with HIGH-DOSE SALICYLATES (ASPIRIN) and EARLY IVIG (steroids only if not responsive)?
KAWASAKI disease
How are coronary artery ANEURYSMS checked for after treatment of KAWASAKI disease?
ECHO
Treatment of this MEDIUM-VESSEL Vasculitis is with HIGH-DOSE SALICYLATES + EARLY IVIG rather than Corticosteroids which would be used only if not responsive to the initial therapy?
KAWASAKI disease
ANCA-associated Vasculitides (granulomatosis with polyangiitis - WEGENER Granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome), Cryoglobulinemic Vasculitis, Henoch-Schönlein Purpura, Cutaneous Leukocytoclastic Vasculitis are all what caliber VASCULITIS?
Small-Vessel VASCULITIS
What is a unique laboratory finding that is common to the SMALL-VESSEL Vasculitides (granulomatosis with polyangiits - WEGENER Granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome)?
They are ALL ANCA-positive (c-ANCA and p-ANCA)
What SMALL-VESSEL Vasculitis is associated with c-ANCA?
Granulomatosis with Polyangiitis (WEGENER Granulomatosis)
What SMALL-VESSEL Vascultitides (2) are associated with p-ANCA?
Microscopic Polyangiitis and Churg-Strauss syndrome
c-ANCA Vasculitis?
Granulomatosis with Polyangiitis (WEGENER Granulomatosis)
A SYSTEMIC, NECROTIZING Vasculitis that primarily affects the RESPIRATORY tract and KIDNEYS?
Granulomatosis with Polyangiitis (WEGENER Granulomatosis) - c-ANCA positive
c-ANCA positive disease with SINUSITIS, NASAL/INNER EAR or LARYNGO-TRACHEAL Inflammation which is HIGHLY-destructive/erosive to CARTILAGE leading to NASAL-SEPTAL perforation and SADDLE NOSE deformity?
Granulomatosis with Polyangiitis (WEGENER Granulomatosis) - c-ANCA positive
Scleritis, Uveitis, Keratitis and an Inflammatory retro-orbital Pseudotumor with muscle dysfunction and PROPTOSIS as well as MONONEURITIS Multiplex can be seen in this c-ANCA positive VASCULITIS?
Granulomatosis with Polyangiitis (WEGENER Granulomatosis)
Cough, Hemoptysis, Pleurisy, CAVITATING lung infiltrates/nodules with diffuse opacities with PULMONARY Hemorrhage as well as a GLOMERULONEPHRITIS WITHOUT IMMUNE-Complexes (“paucy-immune”) associated with c-ANCA positivity?
Granulomatosis with Polyangiitis (WEGENER Granulomatosis)
How is Granulomatosis with Polyangiitis (WEGENER Granulomatosis) - c-ANCA positive disease DIAGNOSED?
Lung/Kidney BIOPSY demonstrating a vasculitis with acute & chronic inflammation
A kidney biopsy that demonstrates a NECROTIZING Focal-Segmental or DIFFUSE GLOMERULONEPHRITIS without SIGNIFICANT immune-deposits (paucy-immune) is associated with what disease?
Granulomatosis with Polyangiitis (WEGENER Granulomatosis) - c-ANCA positive
The presence of “antiproteinase-3 Ab’s” (c-ANCA) in a patient with UPPER airway manifestations, pulmonary infiltrates/nodules and urinary abnormalities consistent with GLOMERULONEPHRITIS is most likely diagnosed with?
Granulomatosis with Polyangiitis (WEGENER Granulomatosis)
How are Granulomatosis with Polyangiitis (WEGENER Granulomatosis) - c-ANCA positive and Microscopic Polyangiitis - p-ANCA positive treated?
HIGH-DOSE Corticosteroids and DAILY Cyclophosphamide (risk of hemorrhagic cystitis and bladder cancer) or RITUXIMAB (instead of cyclophosphamide) FOLLOWED BY WEEKLY Azathioprine or Methotrexate for 18 MONTHS
What is used for PROPHYLAXIS to prevent Pneumocystis Jirovechi infection in patients with HIV or immunosuppressed due to medication therapy?
TMP-SMX
Another ANCA-positive SMALL-VESSEL NECROTIZING Vasculitis that affects the LUNGS and KIDNEYS with rapidly progressive GLOMERULONEPHRITIS or PULMONARY HEMORRHAGE but UNLIKE Granulomatosis with Polyangiitis (WEGENER Granulomatosis) - “c-ANCA positive” this disease is p-ANCA positive (myeloperoxidase Ab’s)?
Microscopic Polyangiitis
What is synonymous with antiproteinase-3 Ab?
c-ANCA (Granulomatosis with Polyangiitis (WEGENER Granulomatosis)
What is synonymous with antimyeloperoxidase Ab?
p-ANCA (microscopic poyangiitis, Churg-Strauss)
What are the ONLY distinguishing factors between Granulomatosis with Polyangiitis (WEGENER Granulomatosis) and Microscopic Polyangiitis?
Granulomatosis with Polyangiitis (WEGENER Granulomatosis) is c-ANCA positive (antiproteinase-3 Ab) whereas Microscopic Polyangiitis is p-ANCA positive (antimyeloperoxidase Ab)
A VASCULITIS that is associated with EOSINOPHILIA and usually follows ASTHMA or ALLERGIC RHINITIS or SINUSITIS and causes EOSINOPHILIC CARDIOMYOPATHY?
Churg-Strauss Syndrome
The ONLY EOSINOPHILIC VASCULITIS (EOSINOPHILS found on BIOPSIES and in BLOOD) and is associated with p-ANCA - “antimyeloperoxidase Ab’s”?
Churg-Strauss Syndrome
How is Churg-Strauss Syndrome treated?
In the SAME manner as Granulomatosis with Polyangiitis (Wegener Granulomatosis - c-ANCA positive) and Microscopic Polyangiitis - p-ANCA positive vasculitides are treated:
HIGH-DOSE Corticosteroids and DAILY Cyclophosphamide (risk of hemorrhagic cystitis and bladder cancer) or RITUXIMAB (instead of cyclophosphamide) FOLLOWED BY WEEKLY Azathioprine or Methotrexate for 18 MONTHS
Seen most OFTEN in patients with HYPERVISCOSITY syndromes, Waldenstrom Macroglobulinemia, Multiple Myeloma and Sjögren Syndrome, these are immunoglobulins that PRECIPITATE in the COLD?
TYPE-I Cryoglobulinemia
Seen most OFTEN in patients with Hep C or HIV and are associated with VASCULITIS, these are rheumatoid factors that are MONOCLONAL IgM or IgA immunoglobulins with specificity for IgG that PRECIPITATE in the COLD?
TYPE-II Cryoglobulinemia
Cryoglobulins associated with RA or SLE are usually of what type?
TYPE-III
LOW C3 and C4 as well as IMMUNE-COMPLEX GLOMERULONEPHRITIS and Cutaneous Purpura are seen with this type of VASCULITIS?
CRYOGLOBULINEMIC Vasculitis
How is MILD Type-II (Hep C/HIV - associated) Cryoglobulinemic Vasculitis treated?
Corticosteroids and Hep C/HIV antiviral therapy (or RITUXIMAB if resistant)
How is SEVERE Cryoglobulinemic Vasculitis (kidney failure, digital gangrene, neurologic disease) treated?
Plasma EXCHANGE
How are the MILD, non viral-associated (Hep C/HIV) Cryoglobulinemic Vasculitides (Types I and III) treated?
Corticosteroids AND Cyclophosphamide (risk of hemorrhagic cystitis and bladder cancer)
A VASCULITIS that occurs mainly in children (like KAWASAKI arteritis) but can affect adults and with GREATER SEVERITY with a purpuric RASH that affects the DISTAL LOWER EXTREMITIES with arthritis, ABDOMINAL PAIN and HEMATURIA?
Henoch-Schönlein Purpura
What must be CAREFULLY checked in patients who are diagnosed with Henoch-Schönlein Purpura even though in MOST cases, it is a self-limited disease?
Kidney function - Progressive Kidney Disease is seen in SOME patients
What is the most likely ETIOLOGY for MEN ≥50 who develop Henoch-Schönlein Purpura?
Solid Tumors or Myelodysplastic Syndrome
Skin BIOPSY of LE RASH revealing a LEUKOCYTOCLASTIC Vasculitis with IgA DEPOSITS is diagnostic for?
Henoch-Schönlein Purpura
Hematuria and Proteinuria or Kidney Disease that develops after a LE purpuric Rash with kidney BIOPSY demonstrating GLOMERULONEPHRITIS with IgA DEPOSITION (IgA NEPHROPATHY) that can progress to PROLIFERATIVE GLOMERULONEPHRITIS is what?
Henoch-Schönlein Purpura
How is Henoch-Schönlein Purpura treated?
Corticosteroids (add CYCLOPHOSPHAMIDE if proliferative glomerulonephritis develops) and evaluate patients ≥50 for underlying malignancy
A VASCULITIS that is seen in patients with SLE, autoimmune disease or certain drugs, Hematologic Malignancies or Systemic Vasculitis that can present with Palpable Purpura, Tender Nodules, Shallow Ulcers or PERSISTENT URTICARIA (≥24 hours) with BIOPSY revealing NEUTROPHILS and MONONUCLEAR Cells invading walls of dermal capillaries with “NUCLEAR FRAGMENTS - dust”?
Leukocytoclastic Vasculitis
How is Leukocytoclastic Vasculitis treated?
Treat underlying disease AND with NSAID’s, Antihistamines (if urticaria - COMBINING H1 and H2 blockers), Colchicine or Dapsone
SLE can manifest frequently with URTICARIAL (leukocytoclastic) Vasculitis, how is this treated in this setting?
Hydroxychloroquine
If a leukocytoclastic vasculitis does not respond to standard treatments (NSAID’s, Antihistamines (if urticaria - COMBINING H1 and H2 blockers), Colchicine or Dapsone or Hydroxychloroquine if due to SLE), how do you treat?
Corticosteroids or with Corticosteroid-Sparing agents Methotrexate or Azathioprine
A MALE 20-40 yo presented with Oral and Genital Ulcers, Uveitis and Vasculitis with THROMBOSIS, BIOPSY reveals neutrophil and mononuclear cell infiltration and associated with HLA-B51?
Behçet Disease
Non-scarring ORAL mucosa or TONGUE ulcers with DEEP, scarring, PAINFUL GENITAL ulcers (vaginal, labial, scrotal) with anterior or posterior uveitis that can cause BLINDNESS, intestinal lesions that mimic Ulcerative Colitis, erythema nodosum, pseudofolliculitis, acneiform nodules and migratory thrombophlebitis are seen with what disease?
Behçet Disease
In this disease, if the skin is pricked with a needle, 48 HOURS later, a pustule-like lesion or papule appears (pathergy)?
Behçet Disease
Rarely, this vasculitis that is associated with HLA-B51 and presents with oral and genital ulcers is associated with CNS involvement of the brainstem, basal ganglia and white matter as well as aneurysms of the arteries of the heart and lungs with hemoptysis?
Behçet Disease
How are MUCOSAL lesions in Behçet Disease treated?
TOPICAL steroids, colchicine and thalidomide
How is OCULAR disease treated in Behçet Disease?
Azathioprine, Cyclophosphamide, Cyclosporine OR INFLIXIMAB AND Corticosteroids
A relapsing and remitting inflammatory disorder of CARTILAGE of the EARS, NOSE and RESPIRATORY TRACT (hoarseness, stridor), OCULAR inflammation (conjunctivitis, episcleritis, scleritis, uveitis, retinal vasculitis or proptosis) and a non-erosive, seronegative polyarthritis, can lead to CAULIFLOWER EAR or SADDLE NOSE, AORTIC/MITRAL VALVE INSUFFICIENCY and is associated with VASCULITIS, MYELODYSPLASTIC CONDITIONS and MALIGNANCY?
Relapsing Polychondritis
How is MILD Relapsing Polychondritis treated?
NSAID’s, Corticosteroids or Dapsone
How is SEVERE (or involving EYES, Respiratory tract) Relapsing Polychondritis treated?
Methotrexate, Azathioprine, Cyclophosphamide, Cyclosporine
A disease that presents ABRUPTLY in those between ages of 16-35 with a DAILY SPIKING HIGH FEVER (102.2ºF) in the evenings, SALMON-COLORED “EVANESCENT” RASH on the trunk and proximal extremities, ELEVATED LFT’s, SPLENOMEGALY and ARTHRITIS (wrists, knees and ankles with joint-space narrowing) with ELEVATED INTERLEUKINS (IL-6, IL-1, IL-18) and ELEVATED TNF-α is called?
Adult-Onset STILL Disease
What unique lab abnormalities are seen in Adult-Onset STILL Disease?
Abnormal LFTs, HIGHLY elevated serum FERRITIN (≥1,000 ng/mL)
What is a potential DEADLY complication that can OCCUR with Adult-Onset STILL Disease and how does it present?
Hemophagocytic Syndrome, presents with PANCYTOPENIA
How is Adult-Onset STILL Disease treated?
Corticosteroids and Methotrexate (if steroids are not enough)
Daily high FEVER SPIKES especially in the evenings, evanescent, salmon-colored RASH on trunk and proximal extremities, ARTHRITIS and elevated serum FERRITIN levels are all seen in?
Adult-Onset STILL Disease
A FAMILIAL autoinflammatory disease that presents typically in Sephardic Jews, Turkish and Armenian people, with intermittent episodes of FEVER, joint pain, abdominal pain and rash? How is it treated? What is the risk?
Familial Mediterranean Fever
Treated with Colchicine
Risk (HIGH) of developing Amyloidosis
Mutations in the GENE for Type-I Collagen causing BRITTLE BONES, BLUE Sclerae and BRITTLE, MISSHAPEN, TRANSLUCENT TEETH with CHILDHOOD FRACTURES that decrease after puberty?
Osteogenesis Imperfecta
How is Osteogenesis Imperfecta diagnosed and treated?
Diagnosed by skin biopsy (analysis of Type-I collagen), treated in MODERATE cases with BISPHOSPHONATES (IV pamidronate)
Mutations in Types-I, III and V COLLAGEN resulting in SMOOTH, HYPEREXTENSIBLE, THIN TRANSLUCENT SKIN, JOINT HYPERMOBILITY, Delayed WOUND Healing with atrophic scarring and EASY BRUISING are all seen in this Syndrome?
Ehlers-Danlos Syndrome
Ehlers-Danlos Syndrome in which MAINLY TYPE-III COLLAGEN is affected (VASCULAR), presents with?
Aneurysms, AV-fistulas, arterial DISSECTIONS with VESSEL, BOWEL and UTERINE (pregnancy) RUPTURE
What should ALL Ehlers-Danlos Syndrome patients be told to AVOID?
Contact sports, Anticoagulants and Unnecessary Surgeries
Mutations in the FIBRILLIN GENE result in abnormal COLLAGEN formation causing OVERGROWTH of LONG BONES (abnormal tall stature), ARM SPAN greater than height, PECTUS EXCAVATUM, LONG DIGITS (arachnodactyly), Dural Ectasia, a HIGH PALATE, MICROGNATHIA, LENS DISLOCATIONS (ectopia lentis), MITRAL VALVE and AORTIC ROOT ANEURYSMS?
Marfan Syndrome
The SKIN and AORTA of this abnormal COLLAGEN syndrome contain abnormally LOW levels of ELASTIN resulting in HYPEREXTENSION and ANEURYSMS which are the leading cause of death?
Marfan Syndrome
Black people aged 20-40 presenting with a multi-systemic disorder characterized by NON-CASEATING GRANULOMAS?
Sarcoidosis
An ACUTE, SELF-LIMITED form of SARCOIDOSIS presenting with HILAR LYMPHADENOPATHY (as is sarcoidosis), Erythema Nodosum and ACUTE ARTHRITIS or TENOSYNOVITIS of the ANKLES (as in sarcoidosis) ± FEVER and ANTERIOR UVEITIS?
Löfgren Syndrome
Symmetric OLIGOARTHRITIS (mostly the LARGE JOINTS of the LE’s - ESPECIALLY the ANKLES with ASYMPTOMATIC LYTIC BONE LESIONS of the HANDS, FEET and LONG BONES with LUNG involvement (pulmonary nodules, fibrosis and infiltrates with NON-CASEATING GRANULOMAS), SKIN involvement (erythema nodosum, lupus pernio - indurated violacous plaques on face and ears), BONE involvement (lytic lesions on hands, feet and long bones), JOINT involvement (arthralgia, polyarthritis), EYE involvement (uveitis), SALIVARY GLAND involvement (parotitis), LYMPHADENOPATHY (hilar and mediastinal), NERVE involvement (brain lesions, peripheral neuropathy, facial nerve palsy, myelopathy), ELEVATED LFTs, HEART involvement (heart block, sudden death, myocarditis), HYPERCALCEMIA and HYPERVITAMINOSIS D are seen with this disease?
Sarcoidosis
ACUTE LE arthritis, ERYTHEMA NODOSUM and SYMMETRIC HILAR LYMPHADENOPATHY?
Löfgren Syndrome (acute, self-limited Sarcoidosis)
When are skin biopsies (not of the erythema nodosum) REQUIRED in diagnosing SARCOIDOSIS or Löfgren Syndrome?
When ACUTE LE arthritis and ERYTHEMA NODOSUM appear WITHOUT SYMMETRIC HILAR LYMPHADENOPATHY
What lab value is elevated in 75% of patients with SARCOIDOSIS but is NOT specific (does not rule in disease)?
Angiotensin Converting Enzyme levels (ACE)
What is SARCOIDOSIS or Löfgren Syndrome treated with ?
Corticosteroids and NSAID’s
What is SARCOIDOSIS or Löfgren Syndrome treated with if corticosteroids are not sufficient?
Methotrexate, Hydroxychloroquine or Colchicine
