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USMLE2 Medicine 04

Rheumatology

QuestionAnswer
4 questions for someone with joint swelling 1. how many joints and which ones?, 2. acute or chronic?, 3. systemic sx's?, 4. e/o joint inflammation?
Polyarticular Arthritis (6) SYMMETRIC poly arthritis! these kids should all go to the same Poly R-ticular High S-S-School. Parvo B19, RA, HepB, SLE, Scleroderma, Sjogren
Monoarticular Arthritis (5) One Goint Hurts So Terribly. OA, Gout/Pseudogout, Hemearthroses, Septic, Trauma
Migratory Arthritis - what is it? inflammation and pain migrates from joint to joint, as prior joint improves
Migratory Arthritis (3) Rheumatic fever, Disseminated N Gonorrhea infection, Lyme disease. LRG. Migrating joints are LaRGe.
Oligoarticular asymmetric arthritis Ankylosing spondylitis, OA of small joints of upper extremities, Polyarticular gout. Oli-Go Articular.
Sjogren syndrome autoimmune disorder in which lymphocytes attack exocrine glands that produce tears and saliva. Arthritis, keratoconjunctivitis sicca (dry eyes, dry mouth), parotid enlargement.
Systemic sclerosis (scleroderma) chronic autoimmune connective tissue disease characterized by skin thickening, vascular alterations, and autoantibodies. Arthritis and Raynaud.
Wegener's granulomatosis form of vasculitis (inflammation of med-sized blood vessels) that affects the lungs, kidneys. Arthritis, URI (sinusitis, rhinitis), lung nodules/hemoptysis, necrotizing glomerulonephritis
What systemic sx's does OA have? none
alpha-Fodrin auto-antigen for Sjogren's Syndrome
clinical signs indicating joint inflammation joint stiffness in the morning>1h, joint erythema and warmth, elevated ESR and CRP
OA is inflammatory or non-inflammatory? non
RA is inflammatory or non-inflammatory? inflammatory
what tests to run on synovial fluid? CCC. cell count, crystals, Cx and Gram Stain
Synovial fluid WBC counts: 200-2K, 5K-50K, >50K 200-2K: OA/DJD and Trauma, 5K-50K: inflammatory, septic arthritis >50K (if before abx given)
needle-shaped negative birefringent gout crystals
rhomboid shaped positive birefringement Pseudogout crystals
anti-ANA (3 dz's) SLE, Sjogren, systemic sclerosis/scleroderma, 5% nl people (low titers)
ANA with peripheral rim pattern SLE
ANA with centromere pattern CREST
ANA with nucleolar pattern systemic sclerosis
CREST limited scleroderma: Calcinosis (Ca deposits in soft tissues - usu fingers knees elbows), Raynaud's, Esophageal dysmotility, Sclerodactyly (thickening of finger and toe skin), Telangiectasia
how to follow disease activity in SLE anti-dsDNA
anti-Smith specific for SLE
anti-histone drug-induced SLE
Anti-Ro (SSA) 1. neonatal SLE, 2. ANA-neg SLE, 3. Sjogren
Anti-LA (SSB) Sjogren
Anti-centromere CREST
Anti-RNP mixed connective tissue disease
Rheumatoid Factor in Rheumatoid arthritis, worse prognosis
anti-CCP CCP=cyclic citrullinated peptide. + in RA
anti-neutrophil-cytoplasmic-Ab ANCA
cANCA (cytoplasmic ANCA) Wegener's granulomatosis
pANCA (perinuclear ANCA) polyarteritis nodosa (PAN) and Churg-Strauss
lupus anti-coagulant, anti-cardiolipin Ab's anti-phospholipid antibody syndrome - hypercoagulable state ass with Ab's directed against phospholipids or cardiolipins
pt with + anti-cardiolipin Ab and +RPR lupus anti-coagulant and anti-cardiolipin Ab's can cause false + VDRL and RPR
2 first trimester spontaneous abortions antiphospholipid Abs (causes spontaneous abortions in otherwise healthy women; also PE and DVT)
hallmark of RA inflammatory synovitis in symmetric distribution --> destroy cartilage and cause bone erosion --> deform joint
risk factor for RA smoking, women 3x more than men
what is the predominant cell type in RA? T lymphocyte
What joints are never involved in RA? DIP's and lower back
Where do rheumatoid nodules occur? areas of mechanical stress (olecranon, occiput, Achilles)
Pt with RA takes methotrexate will cause flare of vasculitis in their rheumatoid nodules
Felty syndrome RA, splenomegaly, neutropenia. He Felt Specially Not RAd.
Caplan syndrome RA, pneumonoiosis
Diagnosis of RA clinical only with supporting xrays and labs - no single test
Treatment of RA NSAIDs (fewer GI side effects) or ASA, glucocorticoids, DMARDs (methotrexate), TNF-alpha inhibitors (infliximab, etanercept)
DMARDs in RA antimalarials, gold, sulfasalazine, methotrexate, TNF receptor inhibitors
What is the side effect of hydroxychloroquine and how to screen for it? retinopathy; regular eye exam
What is the side effect of gold and how to screen for it? rash, dec WBC's, nephritic syndrome. Check renal function, UA, CBC.
When should an RA pt taking gold stop using it? when pruritic rash or proteinuria
What is the side effect of methotrexate and how to screen for it? hepatitis and hepatic fibrosis; pneumonitis; flare rheumatoid nodules. Check CBC and LFT's every 4-8 weeks.
Why should one use a TNF-alpha blocker in RA? TNF-alpha found in large quantities in joints of RA, produced by synovial macrophages and lymphocytes.
if RA pt taking infliximab, what else should they be taking? methotrexate to avoid development of anti-infliximab antibodies
What should pt get before starting anti-TNF therapy? screening for latent TB
In RA pt, what if you see: high RF titers, diffuse rheumatoid nodules, early joint erosions, late age of onset more aggressive disease, worse prognosis
HLA-DR4 in RA pt more aggressive disease, worse prognosis
RA pt has neck pain or paresthesias of hands/feet - what should you rule out? what test to do? atlantoaxial subluxation --> can cause para or quadraplegia. Xray of C-spine. if +, refer to spine surgeon
RA pt with swollen painful calf consider ruptured Baker cyst (extension fo inflamed synovium into the popliteal space)
what environmental factor causes flares in SLE pts? UV-B light
Diagnosis criteria for SLE Need 4 (MD SOAP N HAIR) - Malar or Discoid rash;Serositis (pleuritis, carditis);Oral ulcers;Arthritis;Photosensitivity (UV-B);Neuro (sz, psych);Heme (Coombs + HA, leukopenia, TCP);ANA +;Immune (anti-dsDNA, anti-Smith);Renal (nephrotic, glomerulonephritis)
how is arthritis in RA different from arthritis in SLE? SLE arthritis is non-erosive
if SLE pt has renal involvement, what must they get before starting therapy? renal bx
Libman Sacks endocarditis noninfectious endocarditis seen in lupus (rare)
Best screening test for SLE ANA
Complement levels are ..... in active lupus decreased
How to tx arthritis in SLE? NSAIDs, hydroxychloroquine, corticosteroids
How to tx severe sx's of SLE (lupus nephritis, heart and lung involvement, hemolytic anemia, CNS involvement) cytotoxic agents (azathioprine or cyclophosphamide) and corticosteroids
Fertility rates in SLE nl, but more still births and spontaneous abortion
How to tx repeated spontaneous abortions in SLE pts? anti-phospholipid Ab's --> coagulation --> placental infarcts -->tx with LMWH and ASA during pregnancy
if SLE flare during pregnancy, how to tx? steroids
all pregnant pts with SLE should be screened for what? Anti-Ro --> if cross placenta into baby, can cause neonatal SLE and permanent heart block
drugs that can cause drug-induced SLE it's Quite HIP, Cool n DOPe for drugs to cause SLE: Quinidine, Hydralazine, INH, Procainamide, Chlorpromazine, Methyldopa
how to confirm the diagnosis of drug-induced SLE? once drug is stopped, sx's will resolve in 1 to 2 weeks
Scleroderma sx's Raynaud (vascular damage and dec blood flow to the extremities), skin thickening (accumulation of connective tissue), GI, pulm, renal
Achalasia incomplete LES relaxation, increased LES tone, and aperistalsis of the esophagus (inability of smooth muscle to move food down the esophagus)
GI manifestations of scleroderma esophageal dysmotility, achalasia, small bowel hypomotility, malabsorption, diverticula, dilation of large intestine
Pulm manifestation of scleroderma pulm fibrosis, restrictive lung dz, cor pulmonale; leading cause of death in scleroderma
Renal manifestations of scleroderma. How to tx? scleroderma renal crisis: malignant HTN --> acute renal failure (rapid rise in Cr and proteinuria); tx with ACEi
anti-Scl-70 - in CREST but + in systemic sclerosis
Raynaud's pallor (2/2 vasoconstriction) or cyanosis (deoxygenation of slow-flowing blood) in response to cold or emotional stimuli
how to tx Scleroderma? no cure
how to tx skin manifestations of Scleroderma? D-penicillamine
how to tx skin Raynauds of Scleroderma? CCB
pt with itchy eyes, sandy feeling under their eyes and difficulty swallowing food, increased dental caries, parotid enlargement Sjogren's. eye sx's because of decreased lacrimation --> destruction of corneal epithelium (called keratoconjunctivitis sicca). mouth sx's because of xerostomia.
How to dx Sjogren's Schirmer's test --> decreased tear production; rose Bengal stain --> corneal ulcerations; ANA+, anti-Ro+, anti-LA+
if bx salivary glands of Sjogren's pt, what will you see? lymphocytic infiltration
Tx for Sjogren's? no cure. use eye drops to create artificial tears.
Symmetric poly arthritis with other multisystem involvement S-S-School: SLE, Sjogren's Scleroderma
B-27 (4 associated diseases) Ankylosing spondylitis, Reactive arthritis, Psoriatic arthritis, Enteric arthropathy. PEAR type B27
What 4 characteristics do the PEAR spondyloarthropathies have in common? 1. HLA-B27, 2. ANA and RF neg, 3. involvement of lower back and SI joints, 4. extra-articular manifestations
Ankylosing spondylitis (AS) affects..... axial skeleton and peripheral joints
what age does AS start? teens to 20's
AS affects women or men more? men 3-4x more
man in late 20's presents with chronic lower back pain; morning stiffness, lasting at least 1 hr, improves with exercise AS
which part of the skeleton is usu not affected in AS? cervical spine
3 extra-articular manifestations of AS 1. anterior uveitis, 2. aortic insufficiency --> CHF, 3. 3rd degree heart block
Schober test measures spine flexion. positive test in AS
pt with no more lumbar lordosis AS
bamboo spine and squaring of vertebral bodies AS
how to diagnose AS? xray and clinical
tx for AS NSAIDs, PT, and exercise. If axial disease, TNF-a blockers
Reiter syndrome a reactive arthritis that occurs after a NONgonnococcal urethritis (ureaplasma or chlamydia)
Keratoderma blennorrhagica skin lesions commonly found on the palms and soles but which may spread to the scrotum, scalp and trunk also, and which resemble psoriasis. seen in Reiter syndrome
circinate balanitis The skin around the shaft and tip (glans) penis can become inflamed and scale. seen in Reiter syndrome
arthritis after infectious diarrhea reactive arthritis - usu caused by Campylobacter, Shigella, or Salmonella
Tx for reactive arthritis NSAIDs, PT, and exercise. If axial disease, TNF-a blockers. If caused by chlamydia, then tetracycline x3 wks.
arthritis in DIP joints + pitting of the nails + sausage shaped digits psoriatic arthritis
Pt with UC/Crohn's with arthritis enteropathic arthropathy
pyoderma gangrenosum disease that causes tissue to become necrotic, causing deep ulcers that usually occur on the legs. When they occur, they can lead to chronic wounds. associated with enteropathic arthopathy
erythema nodosum inflammation of the fat cells under the skin (panniculitis). It causes tender nodules that are usually seen on both shins. associated with enteropathic arthopathy
what is the target tissue in OA? destruction of articular cartilage and remodeling/hypertrophy of bone
how is OA different from RA? OA is not inflammatory
what are the most common joints affected in OA? knee, then base of thumb, then hip, then DIP/PIP
joint pain increases with exercise and decreases with rest OA
joint stiffness in AM <20-30 min OA
crepitations in joint OA
what are the systemic manifestations of OA? levels of ESR/CRP? no systemic disease, if ESR/CRP elevated, it's some other process complicating OA or it's not OA
osteophytes and unequal joint space on xray OA
bouchard's nodes osteophytes (bone trying to repair) at the PIP's
Heberden's nodes osteophytes at the DIP's
tx of OA no cure. PT, posture, wt loss. Tylenol, NSAIDs or capsasin cream (deplete nerve endings of substance P) for pain. intra-articular hyaluronic acid injection for refractory cases.
middle-aged man presents with acute, monoarticular arthritis, severe pain waking pt from sleep first presentation of gout (chronic gout can affect multiple joints). looks exactly like cellulitis
tx for gout acute: NSAIDs (indomethacin TID), colchicine (qhr until sx's resolve or until GI upset), steroids if can't use NSAIDs. if no tx, will resolve in 3-14 days. Long term - low purine diet, lifestyle changes (i.e. no EtOH), lower uric acid levels
events that can precipitate gout EtOH, trauma, infxn, steroid wdrawal, HCTZ/furosemide, anti-TB meds, serious illness, surgery
gout is an inflammatory process, so on xray of joint involved in multiple attacks, you would see.... erosive calcifications
how to dx gout analysis of synovial fluid. NOT serum uric acid (of no value)
allopurinol use in acute gout should not be started in an attack, but should also not be discontinued if the pt is on it (avoid fluctuations in uric acid levels to avoid precipitation of crystals)
how to lower uric acid levels for chronic gout probenecid in undersecretors, allopurinol in everyone else - esp with renal failure or kidney stones
pseudogout in pt <50yo think 4 h's - hyPER-PTH, hyPO-phos, hyPO-Mg, hemochromatosis
who gets pseudogout usually? elderly or person with pre-existing joint disease
what is the most common affected joint in pseudogout knee
chondrocalcinosis radiographic evidence of calcification in hyaline and/or fibrocartilage. found in pseudogout
another name for pseudogout calcium pyrophosphate dihydrate deposition disease (CPPD)
tx for pseudogout same as gout - NSAIDs, colchicine
what is the most common cause of septic arthritis N gonorrhea in <40yo; S. aureus in elderly and those with pre-esixting joint disease
when are women at greater risk for septic arthritis menses and pregnancy
Dx a red painful swollen joint arthrocentesis. NOTE that most with gonococcal infxn will have negative cx's and gram stains
how to tx septic arthritis by epidemiology. woman <40 yo use CTX to cover for gonorrhea, elderly person - with nafcillin or vanc to cover for S aureus.
Wegener granulomatosis small vessel vasculitis, mainly affecting resp tract and kidneys, also arthritis
chronic rhinitis that doesn't respond to usual tx and gets worse Wegener granulomatosis
how to dx Wegener's granulomatosis? bx of affected organ (usu nasal septum) - shows vasculitis and granulomas
tx for Wegener's glucocorticoid and cyclophosphamide (immunosuppressive)
PAN will affect most any organ system except.... lungs
pt with malaise, wt loss, abdominal pain, peripheral neuropathy, and foot drop, FOBT+, mononeuritis multiplex polyarteritis nodosa
PAN is associated with what liver disease? active HepB infection
how to dx PAN? bx of involved organ (usu skin, nerves, or muscles) - show vasculitis in medium sized vessels, angiogram of abd vessels (show aneurysms of kidney/GI arteries)
tx for PAN glucocorticoid and cyclophosphamide (immunosuppressive)
Churg Strauss PAN (med vessel vasculitis, any organ) in an asthmatic pt
Cardinal manifestations of Churg Strauss asthma, eosinophilia, lung involvement
middle aged person with new-onset asthma consider Churg Strauss
Mononeuritis multiplex ass w PAN n Churg-Strauss. painful asymmetric asynchronous sensory and motor periph neuropathy; isolated damage to at least 2 separate nerve areas. Multiple nerves in random areas of the body. More advanced: less multifocal and more symmetric.
tx of Churg Strauss glucocorticoid (prednisone) and cyclophosphamide (immunosuppressive)
dx Churg Strauss bx of affected tissue - med vessel vasculitis
Temporal Arteritis (giant cell arteritis) - large artery vasculitis affecting head, eyes, optic nerve
new-onset HA in someone >50yo consider temporal arteritis
untreated temporal arteritis permanent vision loss
HA and pain in uni/BL temples, scalp tenderness temporal arteritis
jaw claudication temporal arteritis
polymyalgia rheumatica proximal stiffness - neck arms hips. associated with temporal arteritis
First test in someone suspected of TA ESR is always increased in temporal arteritis. 100% sensitive!
Dx TA ESR with bx confirmation of temporal artery showing giant cells
tx TA if ESR elevated, immediate corticosteroids - even before the bx!
what is never involved in inflammatory myopathy? ocular muscles. this distinguishes myopathy from myesthenia gravis and Eaton Lambert Syndrome
lifting objects, combing hair, getting up from chair proximal muscle weakness. consider a myopathy (poly, dermato, inclusion body myositis).
proximal muscle weakness, diffuse lilac rash of sun-exposed areas, scaly lesions over knuckles (Gottron's papules) dermatomyositis
what labs to order if suspect inflammatory myopathy? CPK and aldolase
anti-Jo-1 inflammatory myopathy (poly, dermato, body myositis)
Dx inflammatory myopathy. Bx of muscle. EMG (helpful, but not required) shows short duration, low amplitude
how to tx myositis if poly or dermato myositis, then use steroids. inclusion body myositis is resistant to immunosuppressive therapy.
Created by: christinapham