Rheumatologic Precision & Pearls #1 Flashcards
Fibromyalgia is a disorder in which the patient has abnormal pain perception. It is MC in _______ and is characterized by
Women ages 20-55
Chronic, widespread MSK pain in 11 of 18 points and pain for 3 or more months
Sleep studies in a patient with fibromyalgia shows…
no REM sleep cycle
Treatment for fibromyalgia
-Conservative: sleep hygiene, low impact exercise
-Amitriptyline (1st line)
-SSRI’s
-Pregabalin (FDA approved for Fibro)
Gout is ______ deposition in a joint or bone. 90% of this condition occurs in ____.
Name some triggers for gout.
uric acid
90% in men
-Purine rich foods (beer, yeast, alcohol, seafood, liver)
-Medications: Thiazides, Loop diuretics, ACE Inhibitors, ARBs (not Losartan), Aspirin, Ethambutol
What are the symptoms of gout
-Podagra (first MTP joint of the great toe)
-Lower extremity MC affected
-Erythema, pain, warmth, tenderness, fever
Initial diagnostic for gout and what does it show?
What do the radiographs of a gouty joint show?
Arthrocentesis: negatively birefringent needle shaped crystals
Radiographs: mouse or rat bite lesions (punched out erosions with sclerotic and overhanging margins)
Increased WBC, Primarily neutrophils
For an acute gout attack, what is the treatment?
-NSAIDs
-Corticosteroids if unable to take NSAIDs (renal problems)
-Colchicine last line
For chronic gout, what should you do?
Lifestyle changes: diet, no alcohol
Allopurinol (xanthine oxidase inhibitor) or Febuxostat = both decrease uric acid production
Gout is a problem with the metabolism of what type of molecule?
In gout, what compound precipitates into the synovial fluid?
Purine
Sodium Urate
Pseudogout, also known as Calcium Pyrophosphate Dihydrate Deposition Disease (CPPD), is the deposition of _____ in the joint. It occurs MC in what joint? Even though it mimics gout symptoms, what does the arthrocentesis show in this condition?
calcium pyrophosphate dihydrate
MC in Knee
Positively birefringent rhomboid shaped crystals
Treatment for pseudogout
-Intraarticular steroids if 1-2 joints
-NSAID if > 2 joints
-Colchicine an option as well
Polymyositis is an inflammatory disorder of striated muscle involving ____________. Explain symptoms of this condition.
proximal limbs, neck, and pharynx
-Progressive symmetric proximal muscle weakness (shoulders, hips) - combing hair, rising from chair, etc.
-Dysphagia, fever, weight loss
-Decreased muscle strength
-NO RASH!
What diagnostics can be done for polymyositis and what do they show?
-Increased CK and aldolase
-Antibodies: Anti-Jo 1, anti signal recognition protein (most specific)
What is the most specific marker for polymyositis?
Anti-signal recognition protein
Anti Jo-1 as well is included in this diagnosis
What is the DEFINITIVE diagnostic for polymyositis?
Muscle biopsy = endomysial inflammation
Treatment for polymyositis
High dose corticosteroids
-Methotrexate if no response to steroids
On the other hand, what is dermatomyositis?
Explain the symptoms of this condition
Polymyositis + skin involvement
-Progressive symmetric proximal muscle weakness
- Decreased muscle strength
-Skin:
–Heliotrope rash: edema/blue eyelids
–Gottron’s Papules: on knuckles
–Shawl Sign: rash of upper back, shoulders, and chest
Diagnostics for dermatomyositis are similar to that of polymyositis. Name them and tell what you find including the tumor markers
-CK and aldolase are elevated (muscle enzymes)
-Muscle biopsy = definitive
-Anti Jo-1 and Anti-Mi 2
True or False: Dermatomyositis has an increased risk of malignancy
True
25% of cases are associated with cancer
Treatment for dermatomyositis
-High dose glucocorticoids
-Methotrexate, Azathioprine, IVIG are other options
Polymyalgia Rheumatica is inflammation of the joints, bursae, and tendon. It is HIGHLY associated with what other condition?
What are the symptoms of PMR?
GCA (Temporal arteritis)
-Pain and stiffness in proximal muscles and joints
-NORMAL muscle strength
-May have decreased ROM
Furthermore, to differentiate PMR from polymyositis, what do the labs show?
PMR:
-Normal muscle enzymes
-Markedly elevated ESR and -CRP
-Increased platelets (acute phase reactants)
What is the treatment for PMR?
Low dose corticosteroids, or Methotrexate
Reactive arthritis, also known as Reiter Syndrome, may be seen 1-4 weeks after _________
However, it also has a high association with….
Chlamydia or GI infection
HLA-B27 positivity
Name the triad of symptoms associated with reactive arthritis as well as other symptoms of this condition.
-Arthritis + urethritis + conjunctivitis (can’t see, can’t pee, can’t climb a tree)
-Lower extremity MC affected
-Oral lesions
-Keratoderma blennorrhagicum: hyperkeratotic lesions on palms and soles
Treatment for Reactive Arthritis
NSAIDs
-Methotrexate if needed
-ABX if chlamydia related
Pathophysiology of Rheumatoid Arthritis
What are some risk factors?
Chronic, progressive cartilage destruction and joint structure loss due to destruction by pannus. T Cell mediated
Females, smoking
Symptoms of Rheumatoid Arthritis
-Morning stiffness > 1 hour, improves throughout the day
-MC affects small joints (wrist, MCP, PIP, spares the DIP)
-Symmetrically inflamed joints, warm boggy
-Ulnar deviation of the hand at MCP joint
-Swan neck/Butonniere deformities
-Nodules over bony prominences
Regarding RA, what is Felty Syndrome? What is Caplan Syndrome?
Felty Syndrome: RA + neutropenia + splenomegaly
Caplan: RA + pulmonary nodules + pneumoconiosis
Labs (which is the most specific) for RA.
What do XR’s show?
Rheumatoid Factor positive
Anti-CCP (most specific)
XR: symmetric joint narrowing, osteopenia, erosions, C1-C2 subluxation if the spine is affected
Treatment for RA
Which is the first line for symptom control?
Which drug is safe in pregnancy?
-DMARDs: Methotrexate, Leflunomide (first line), + NSAIDs
NSAIDs
Hydroxychloroquine
Methotrexate drug facts (MOA and adverse effects)
Folic acid antagonist
Hepatitis, bone marrow suppression, interstitial pneumonitis
What is the MC type of Juvenile Idiopathic Rheumatoid Arthritis?
What are the symptoms?
Still’s Disease (Systemic):
-Daily spiking fevers
-Polyarthralgias
-Salmon colored migratory rash at night
Treatment for JRA?
NSAIDS, PT
-Anakinra, Methotrexate are also options
Lupus (Systemic Lupus Erythematosus) is a chronic multi-organ disorder of connective tissues. It is also a Type _____ hypersensitivity reaction. What are some risk factors for Lupus/SLE?
Type III
Female, sun exposure, African American, estrogen use
Symptoms of SLE including the triad, as well as other systemic symptoms
Triad: joint pain + fever + butterfly malar rash
-Constitutional symptoms, oral ulcers, pleuritis
-If discoid in nature: annular patches on face that heal with scarring
What labs are drawn for SLE and what are the specific ones for this condition?
-ANA (initial)
-Anti double stranded DNA and Anti Smith
-Antiphospholipid antibodies = increased risk of arterial and venous thromboses
-Decreased complement levels (C3 and C4) mark progression of disease
Treatment for SLE
-Skin: Sun protection, Hydroxychloroquine for lesions
-Arthritis: NSAIDs or Acetominophen
-+/- pulse-dose corticosteroids
-Methotrexate, Belimumab if no response to other treatments
Drug Induced Lupus is SLE caused by drugs such as
Quinidine, Hydralazine, Isoniazid, and Procainamide
What labs are drawn for drug-induced SLE?
Positive ANA
Anti-histone antibodies (hallmark)
Antiphospholipid syndrome is a disorder of thromboses due to antibodies against negatively charged phospholipids. This results in what symptoms?
-Increased risk of thromboses: DVT, PE, recurrent miscarriages, atherosclerosis, stroke, TIA, cognitive deficits
What diagnostics are done to prove the patient has antiphospholipid syndrome?
-Lupus anticoagulant: increased PTT
-Failure to correct PTT with mixing studies
-Prolonged Russell Viper Venom Test: most specific
Treatment for antiphospholipid syndrome
-Lifelong Warfarin Therapy or LMWH for pregnant
Scleroderma (Systemic Sclerosis) is a connective tissue disorder in which collagen deposition leads to fibrosis of skin and internal organs. Explain what CREST (Limited) is and what Diffuse types are.
-Limited (CREST): tight, shiny, thick skin on face, neck, and distal to knees and elbows. Spares trunk.
–Calcinosis Cutis: calcium spots under skin
–Raynaud’s
–Esophageal motility disorder
–Sclerodactyly
–Telangiectasias
Diffuse: shiny, thick skin of trunk and proximal extremities. Greater organ involvement.
What labs are specific to scleroderma?
Anti centromere antibodies (CREST)
Anti SCL 70 antibodies & anti-topoisomerase (diffuse)
ANA positive
Sjogren Syndrome is an autoimmune disorder of the ________ (_____)
Symptoms of this condition include
Exocrine glands (salivary and lacrimation)
-Dry mouth (xerostomia), dry eyes, dry vagina (dyspareunia)
-Parotid gland enlargement
-Dental caries
What diagnostics are done for Sjogren’s (which labs are specific, what eye test is done, and what is definitive?)
Labs: AntiSS-A (Ro) and AntiSS-B (La)
Schirmer Test: <5mm after 5 minutes
Lip or Parotid Biopsy: definitive
Treatment for Sjogren’s Syndrome
-Increase fluid intake
-Use of artificial tears
-Pilocarpine or Cevimeline: cholinergic drugs that increase lacrimation and salivation
Pilocarpine increases salivation and lacrimation. However, what are some adverse effects of this medication?
Diaphoresis, bradycardia, flushing, diarrhea, nausea, vomiting, blurriness
What’s one complication of Sjogren’s (in other words, what does it increase the risk of?)
Non-Hodgkin Lymphoma
Behcet’s Syndrome is characterized by
Recurrent, painful oral and genital ulcers, erythema nodosum, uveitis/conjunctivitis, arthritis, and CNS involvement (can mimic MS)
What population is at increased risk for Behcet’s Syndrome?
What diagnostic is definitive?
What’s the treatment?
Asians, Mediterranean, Middle Eastern
Biopsy
Corticosteroids
Takayasu Arteritis is a large vessel vasculitis that affects
What diagnostic confirms this
aorta, aortic arch, and pulmonary arteries
Angiography
What are some symptoms of Takayasu Arteritis?
-LE claudication
-HTN crisis
-TIA/Stroke
-Asymmetric BP between arms
-Decreased pulses
-Bruits
-Arm claudification
Think of all the arteries and symptoms that would affect them
Treatment for Takayasu
High dose corticosteroids
What is Kawasaki Syndrome?
Small and medium vessel vasculitis involving the coronary arteries
Kawasaki Syndrome MC occurs in what population?
What are the symptoms of this condition (think warm).
Children <5, Asian, boys
Warm + CREAM
–Fever >5 days + 4/5 of:
—Conjunctivitis
—Rash
—Edema/Erythema
—adenopathy (cervical)
—Mucositis (strawberry tongue)
Treatment for Kawasaki Syndrome
-IVIG + Aspirin
Eosinophilic Granulomatosis with Polyangitis can be a rare side effect of which two medications?
What is the triad of symptoms with this condition?
Montelukast and Zafirlukast
Asthma + Eosinophilia + Chronic rhinosinusitis
What labs are seen with Eosinophilic Granulomatosis with Polyangitis, what is the definitive diagnostic and treatment?
P-ANCA positive + Eosinophilia
Biopsy = definitive
Glucocorticoids + Cyclophosphamide
Granulomatosis with Polyangitis (GPA - Wegener’s) is small vessel vasculitis of …..
This means the triad of symptoms with this condition is…
Nose, lungs, and kidneys
-Upper Respiratory tract involvement (nasal congestion, otitis media) + lower respiratory tract involvement (cough, wheezing, hemoptysis) + glomerulonephritis (rapidly progressive with RBC casts)
What labs are seen with GPA, what is definitive, and what is the treatment?
C-ANCA positivity
Biopsy
Glucocorticoids + Cyclophosphamide
Microscopic Polyangitis (MPA), much like GPA, is small vessel vasculitis. However, what is the difference?
No naspopharyngeal symptoms and does affect the capillaries
Symptoms of MPA
-Fever, palpable purpura
-Rapid acute glomerulonephritis with RBC casts
Diagnostics for MPA
Treatment
-P-ANCA positive
-Biopsy definitive
Glucocorticoids + Cyclophosphamide
Immunoglobulin A Vasculitis (Henoch-Schonlein Purpur) is an Ig-A mediated small vessel vasculitis. This occurs MC in what population? What precipitates it?
90% in kids
After infection (URI, GABHS, Parvovirus B-19)
Symptoms of HSP
HSPA
-Hematuria
-Synovial (arthritis or arthralgia)
-Palpable purpura
-Abdominal pain (GI bleed?)
A kidney biopsy is definitive for IgA vasculitis (HSP), what is shown on it?
What is the treatment?
Mesangial IgA deposits
Supportive
Anti-GBM Antibody Disease (Goodpasture’s Disease) is IgG antibodies against Type IV collage of alveoli and glomerular basement membrane of kidneys. What are the symptoms?
Only in lungs and kidneys
–Hemoptysis
-Cough
-Hematuria
-Oliguria
UA for Anti-GBM shows:
What antibodies are present?
What is definitive and what is seen?
RBC casts
Anti-GBM antibodies
Linear IgG deposits
Treatment for Goodpasture’s
Gluco + Cyclophosphamide + Plasmapheresis
Psoriatic Arthritis is associated with _______ and what labs?
HLA-B27 positivity
RF -, ANA -
Name some symptoms of psoriatic arthritis
-Arthritis
-Dactylitis
-Sacroilitis
-Chronic Uveitis
-Psoriasis
What do radiographs of psoriatic arthritis show?
Pencil in cup deformities: thin end of one bone being inserted into a thicker bone
What is the treatment for psoriatic arthritis?
-Mild: NSAIDs
-Moderate: Methotrexate
-TNF Inhibitors (-mab): if no response
