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USMLE2 Medicine 04
Rheumatology
Question | Answer |
---|---|
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. |