Rheum Flashcards
What does RA cause?
A deterioration of joints from inflammation
What are key initiators of RA pathogenesis?
T lymphocytes
What is the shared epitope among patients with RA?
HLA-DRB
What are the clinical manifestations of RA?
Synovitis
Rheumatoid Nodules
Joints and specific organs are affected
Cardiac complications
What area of the body is never involved in RA?
Lower back
Is RA mono or poly -articular?
Polyarticular
What is not involved in RA?
trauma to the joints
Are joints symmetrically or asymmetrical affected in RA?
Symmetrically however, it may start as asymettrical
What are rheumatoid nodules?
SQ nodules that are present on pressure points
What joints are effected in RA?
Cervical Spine Shoulders Elbow Hand and Wrist Hip Knee Foot and Ankle
What organs are effected by RA?
Lungs --> excess fibrous CT Eye Skin Heart --> pericardial effusion Nervous System Blood --> hypochromatic-microcytic anemia
What are some cardiac complications of RA?
MI Disambiguation Stroke Atherosclerosis Pericarditis Endocarditis Left Ventricualr Heart Failure Vasculitis
What is the screening test for RA?
ANA positive
What is RF negative RA called?
Seronegative
Which RA autoantibody is more diagnostic of RA?
Anti-CCP
What is Domain 1 of RA diagnostic criteria?
Joint Involvement
Max of 5 points
1 md. - lg. joint --> 0 2-10 md. to lg --> 1 1-3 small --> 2 4-10 small --> 3 > 10 small --> 5
What is domain 2 of RA diagnostic criteria?
Serology
Max of 3 points
Neg. Rh and Anti-CCP –> 0
One positive at low titer, < 3X normal –> 2
One positive at high titer, > 3X normal –> 3
What is domain 3 of RA diagnostic criteria?
Duration of synovitis
Max 1 point
< 6 weeks –> 0
> 6 weeks –> 1
What is domain 4 of RA diagnostic criteria?
Acute phase reactants
Max 1 point
Neg. CRP or ESR –> 0
Abnormal CRP or ESR –> 1
What is the diagnostic Criteria for RA?
patient must get 6 points in order to definitvely diagnosis RA
What is Felty Syndrome?
Triad of:
RA
Neutropenia
Splenomegaly
What is Polymyalgia Rheumatica?
Pain in proximal muscles
What are the clinical presentations of Polymylagia Rheumatica?
General aches and pains and stiffness
“trouble getting dressed”
Pain greater in the morning
Strength is intact but it will exacerbate pain
What is the best test for diagnosing Polymyalgia Rheumatica?
ESR
What is the treatment for Polymylagia Rhematica?
Oral Prednisone in high doses
Temporal artery biopsy
What causes Reactive Arthritis?
Occurs post infection with Chlamydia, Campylobacter, Salmonella, Shigella, and Yesinia
What is the classic Triad of Reactive Arthritis?
Noninfectious Urethritis
Arthritis
Conjunctivitis
How do you treat Reactive Arthritis?
Treat symptoms and the underlying infection:
Antibiotics
NSAIDs
Corticosteroids
DMARDs –> only if NSAIDs and corticosteroids are ineffective; methotrexate or SULFASALAZINE (for GI)
What is anterior Uveitis?
Red Eye Pain worsens when reading Progressive and occurs over a few hours Blurred vision Photophobia Excess tear production Abnormally shaped pupils
What are the clinical manifestations of Juvenile Idiopathic Arthritis?
< 16
Arthritis in one or more joints
Duration of disease > 6 weeks
Joint inflammation, contractures, and joint damage
Periods of no symptoms and then quick onset of symptoms
What is Systemic Onset JIA?
Repeated fevers of 103 with fluctuating slamon collor rash
Inflammation of the intenral organs and joints
Anemia
Leukocytosis
What is polyarticular JIA?
Arthritits in 5 or more joints with major symptoms of pain in the knees, ankles, wrists, and fingers
Constant pain
What is Oligoarticular JIA?
Arthritis in 4 or fewer joints withint the first 6 months of onset
50% of kids
F>M
Those diagnosed before age 7 have the best change of disease subsiding
High risk of uveitis
Large joints are effected
What is enthesis related JIA?
Involves ligaments as well as the spine
Aka Spondyloarthritis
What are the diagnostic tests for JIA?
Positive ANA
Synovial fluid Class II inflammatory
X-rays show soft tissue swelling, periarticular osteoporosis, growth disturbances, and loss of joint space
What are the three Inflammatory Myopathies?
Polymyositis
Dermatomyositis
Inclusion Body Myositis
What are the clinical manifestations of Polymyositis?
Proximal muscle weakness and upper and lower limbs bilaterally
Difficulty raising arms, lifting objects, or combing hair
Trouble climbing stairs and getting up from sitting
Systemic Symptoms:
GI –> dysphagia, bloating, and constipation
Cardiac –> arrhythmias and condition defects
Pulmonary –> Aspiration pneumonia, INterstitial lung disease, and bronchiolitis
Renal –> ATN
What diagnostics are abnormal in Polymyositis?
Leukocytosis
Elevated CK and LDH
ANA and myositis antibodies
What is the treatment for Polymyositis and Dermatomyositis?
There is no cure!
Corticosteroids for inflammation –> Prednisolone 1mg/kg/day for 4-8 weeks
Monitor CK levels and muscle strenght
Immunosuppresive agents --> Only give these if there is no repsone to steroid within 4 weeks of treatment or if there are extra-skeletal manifestations Azathioprine Cyclophosphamide Chlorabucil Cyclosporine
IVIG INflixumab Rituximab Diet Consults
What are the clinical manifestations of Dermatomyositis?
Heliotrope Rash –> purple discoloration on upper eyelids, flat red rash on cheeks and upper trunk
Grotton’s papules –> raised violaceous scaly rash on knuckles
Dilated capillary loops at the base of the fingernails
irrgular cuticles
“dirty” horizontal lines that resemble mechanics hands after a day of work
What does a muscle biopsy reveal in patients with Dermatomyositis?
Perivascular and perimysial inflammation
What are the clinical manifestations of Inclusion Body Myositis?
Asymmetrical weakness
Falling is common due to quadriceps weakness and artophy
Facial muscle weakness but not ocular
Weakness and atrophy of distal muscles, especially the foot extensors and deep finger flexors
Cannot tie a knot or hold a gold club
Dysphagia and choking
Progresses slowly over the years
What is the treatment for Inclusion Body Myositis?
Resistant to Immunotherapy so give –>
Azathioprine + Prednisolone for a few months
What are tophi?
Deposits of uric acid crystals into tissues
What are the risk factors for Gout?
Men Women after menopause People with kidney disease Obesity HTN Hyperlipidemia Diabetes Family History
What is primary Gout?
inherited
What is secondary gout?
acquired
What does underexcretion cause in gout and what is it caused by?
increased uric acid levels in the blood
Causes by clinical renal disorders like renal insufficiency or a decreased GFR
What is overproduction in gout caused by?
Excessive dietary purine intake from red meat, organ meat, or shellfish
Alcoholic beverages especially beer
How do we diagnose Gout?
Arthrocentesis –> will show intracellular uric acid crystals with negative birefrinence under polarized light microscopy
Elevated serum uric acid levels > 6.8 –> not if only finding though
24 hour urine –> underexcretors with have normal levels and overproducers will have elevated levels
What are common trigger point areas of Fibromyalgia?
Occiput Low cervical Trapezius Supraspinatus 2nd rib Lateral epicondyle Medial fat pad of the knee Greater trochanter Upper glute
What diseases are large vessel vasculitis?
Giant Cell (temporal) arteritis Takayasu Arteritis
What are the effects of large vessel vasculitis?
Limb claudication Assymetric blood pressures Absence of pulses Bruits Aortic dilation
What diseases are medium vessel vasculitis?
Polyarteritis Nodosa (PAN) Kawasaki
What are the effects of medium vessel vasculitis?
Cutaneous nodules Palpable purpura Livedo reticularis Digital gangrene Mononeuritis multiplex Acute foot/wrist drop Microaneurysms Abdominal pain Decreased appetitie
What disease are small vessel vasculitis?
Microscopic Polyangitis (MPA) Granulomatosis with Polyangitits (Wegener's) IgA Vasculitis (henoch-schonlein)
What are the effects of small vessel Vasculitis?
Palpable purpura Vesiculobullous lesions Urticaria Glomerulonephritis Alveolar hemorrhage Splinter hemoorhahes Uveitis Episcleritis Scleritis Cutaneous extravascular necrotizing granulomas
What is the diagnostic criteria for Temporal Arteritits?
presence of 3/5 of the following:
> 50 at onset
New onset localizaed headache
Tenderness or decreased pulse of temporal artery
ESR > 50
Biopsy revelas necrotizing arteritis with predominance of mononuclear cells or a granulomatosus process with multinucleated giant cells
What is the diagnostic criteria for Polyarteritis Nodosa (PAN)?
3 of the following:
Otherwise unexplained wt. loss greater than 4 k LIvedo reticularis Testicular pain or tenderness Myalgias (excluding hip or shoulder) Weakness of leg muscles Tendnerss of leg muscles New onset diastolic BP > 90 Elevated levels of BUN Biopsy of small or medium sized arteries contains polymorphonuclear cells Mononeuropathy or polyneuropathy
What is the diagnostic criteria for Kawasaki’s?
Presence of fever > 5 days wihtout any other explaination with at least 4/5 of the following:
Bilaterally bulbar conjunctival injection
Oral mucous changes, including injected pharynx or fissured lips, or strawberry tongue
Peripheral extremity changes including erythema of palms/soles, edema of hands and feet, and periungal desquamation
Polymorphous rash
Cervical LAD
What is the diagnostic criteria for IgA Vasculitis?
MANDATORY TO HAVE PURPURA WHICH IS PALPABLE AND IN CLUSTERS
Must have one or more of the following:
Abdominal pain that is usually diffuse with acute onset
Arthritis or arthralgia that is acute onset
Renal involvment of proteinuria or hematuria
Leukocystoclastic vasculitis or proliferative glomerulonephritis with predominant immunoglobulin A deposition
What are the constitutional symptoms of SLE?
FATIGUE
fever
LAD
UNintentional wt. loss or gain
What are the skin/Rash symptoms of SLE?
Malar rash Discoid rash Bullous lesions patchy or diffuse alopecia Photosensitivity Panniculitis Palpable purpura Raynaud's Livedo reticularis Childbain lupus
What are the ocular symptoms of SLE?
Conjunctivitis Sicca symptoms Photophobia Blindness Retinal Vasculitis Optic Neuritis
What are the MSK symptoms of SLE?
Symmetrical arthritis Polyarthritis Nonerosive arthritis Jaccoud's arthropathy Myalgia AVN
What are the heart symptoms of SLE?
Pericarditis Myocarditis Premature atherosclerosis Conduction system abnormalities Libman-Sacks endocarditis
What are the lung symptoms of SLE?
Dyspnea or pleuritic CP suggestive of pleuritis
Pneumonitis
Pulmonary HTN
Diffuse alveolar hemorrhage
What are the GI symptoms of SLE?
Peritonitis Hepatitis Pancreatitis Mesenteric vasculitis Intestinal pseudo-obstruciton
What are the GYN symptoms of SLE?
history of miscarriages
What are the kidney symptoms of SLE?
Proteinuria LE edema HTN Glomerulonephritis ARF Interstitial nephritis Antiphospholipid nephropathy
What are the blood symptoms of SLE?
Anemia of chronic disease
Leukopenia
Thrombocytopenia
Arterial/venous thrombosis
What are the neuropsych symptoms of SLE?
Seizures Headaches Psychosis Cognitive dysfunction Peripheral Neuropathy Myelopathy
What are the four types of mucocutaneous lupus lesion patterns?
Acute cutaneous lupu Erytehmatous (ACLE)
Subacute cutaneous lupu Erythematosus (SCLE)
Chronic Lupus Erythematosus (CCLE)
Nonspecific
What rashes are included in ACLE?
MALAR RASH = butterfly rash
maculopapular rash = areas that are photosensitive
Non-scarring alopecia
What rashes are included in SCLE?
papulosquamous or annular
Scaly
Psoriaform
Most photosensitive
Occurs on torso and limbs and spares face
Anti-Ro and Anti-La positive
Induced by medications like HCTZ or terbinafine
What rashes are included in CCLE?
Discoid lupus = raised, erythematou placques with adherent scale
Lupus panniculitis = deep, firm nodules with predilection for scalp, face, arms, buttocks, and thigh
Childbain lupus that resembles frostbite
What are the nonspecific rashes that occur in SLE?
Vasculitis Purpura Livedo Reticularis Raynaud's Alopecia Urticaria Nail-fold periungal capillaries Bullous lesion
What is the diagnostic criteria for SLE?
SOAP BRAIN MD
Must have presence of 4 of these criteria
What is included in Serositis?
Pleuritis
Pericarditis
patients are 40X higher risk of MI and accelerated atherosclerosis
What is Jaccoud’s arthroplasty?
patients with long standing SLE and they have joint deformities like Boutonniere’s or Swan neck but on x=ray there are no bony erosions
It is due to ligament laxity
What blood disorders are common with SLE?
Hemolytic anemia with reticulocytosis
Leukopenia < 4000 on > 2 occasions
Lymphopenia < 1500 on > 2 occasions
Thrombocytopenia < 100,000 on > 2 occasions in the absence of offending drugs
Get COOMBS workup
What renal disorders are common with SLE?
Persistent proteinuria > 0.5 grams/day or > 3+ on dipstick
Urine exam will show cellular casts that are either RBC, HgB, Granular, tibular, or mixed
What is the optic diagnostic tool for lupus nephritis?
Renal biopsy
What is the most frequent cause of death of patients with SLE?
Nephritis
Serum Creatinine > 2.4
Which immunologic tests are best to diagnose SLE?
ANTI-dsDNA
ANTI-SM
COmplement = disease activity they will be low
What neurological diseases are associated with SLE?
Seizures
Psychosis
What drugs cause drug induced lupus?
Hydralazine Quinidine Carbamazepine Isoniazid Methyldopa Minocycline Chlorpromazine Procainamide Phenytoin INfliximab Etanercept Adalimumab Simponi Cimzia
What is en coupe de sabre?
linear patch of scleroderma on scalp/face
What is localized scleroderma?
Morphea - single or multiple patches of hard skin form on the trunk especially in kids and spontaneously disappear
Linear scleroderma - linear bands of hard skin across face, single arm, or leg
What is sytemic Scleroderma?
Limited Cuteanous =80% of patients; CREST
Diffuse cutaneous = 20%; internal organs
What are the phases of Sclerodactyly seen in scleroderma?
Edematous stage = shiny and loose skin folds
Inflammatory stage = Thickening continues; destroys normal sweat glands; intense itching
Atrophic Stage = very tight, joint contractures causing hands to come in
What are the diagnostic criteria for Scleroderma?
Patient must have one major criteria or > 2 minor criteria:
Major –> Proximal scleroderma
Minor –> Sclerodactyly, digital ischemia with digital pitting scars or atrophy of finger pads, or bibasular pulmonary fibrosis
What is the number 1 disease to kill patients with scleroderma?
lung disease
Which type of scleroderma has worst prognosis?
Diffuse
What is the pathogenesis behind Sjogren’s Syndrome?
Unknown trigger leads to vascular endothelial cells in the exocrine glands in genetically predisposed patients
Lymphocytes are attracted into the gland and are activated
Cytokines are released
Gland inflammation and destruction occur causing the sicca symptoms and parotid swelling
What is primary Sjogrens?
Keratoconjunctivitus sicca and xerostomia
What is secondary Sjogren’s?
Complication of anotehr systemic autoimmune disease like SLE, RA, or primary biliary cirrhosis
What is the diagnostic criteria for Sjogren’s?
Ocular staining score of at least > 3
Biopsy of salivary glands showing lymphocytic sialadenitits
Positive Anti-Ro or Anti-La OR positive RF and Positive ANA
Patients must have 2 citeria
What is Hypertrophic Arthritits?
Characterized by bone formation at the site of involved joint
Contained within the bone = subchondral sclerosis
Protrudes from the parent bone = Osteophytes
What is erosive arthrittis?
RA
Indicates underlying inflammation
Characterized by tiny, marginal, irregularly shaped lytic lesions in or around the joint surfaces
What is infectious arthritis?
Characterized by joint swelling, osteopenia, and destruction of long, contiguous segments of the articular cartilage
What is primary degenerative arthritis?
Occurs on weight bearing joints such as hips, knees, humerus, feet, and hands
What is secondary degenerative arthritis?
Post-traumatic infection
What is erosive degenerative arthritis?
Associated with inflammation and synovial proliferation
What are modifiable risk factors of osteoarthritis?
These cause susceptible joint
Muscle strength of quadriceps femoris Physical activity/occupation --> constant use Joint injury --> ACL tear increases risk Joint alignment Leg length inequality
What is the strongest predictor of knee osteoarthritis progression?
Knee mal-alignment
What are Modifiable Systemic Risk Factors for OA?
Obesity
Diet
Bone Metabolism
What are non-modifiable risk factors of OA?
Age –> older
Sex –> WOmen
Genetics
Ethnicity
What are the risk factors for Infectious Arthritis?
IV drug use Chronic Steroid use Joint replacement Recent joint trauma Diabetes
