Term
chronic systemic, multi organ autoimmune d/o of connective tissues. Type III HSN |
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Definition
systemic lupus erythematosus (SLE) |
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Term
Systemic lupus erythematosus (SLE) usually happens in... |
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Definition
young females
onset in 20s-40s
increased in AA, hispanic, native americans |
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Term
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Definition
genetic
environmental sun exposure
infections
hormonal (estrogen) |
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Term
Drugs that cause drug induced SLE |
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Definition
procainamide
hydralazine
INH
quinidine
+ anti-histone bodies
drug induced lupus usually resolves w/ drug d/c |
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Term
Clinical manifestations of SLE |
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Definition
- triad of joint pain (90%), fever, & malar "butterfly" rash (cheeks & bridge of nose - sparing nasolabial folds) Serositis: pericarditis, pleuritis
- discoid lupus: annular, erythematous patches on face & scalp heals w/ scarring
- systemic: CNS, cardio, glomerulonephritis, retinitis, oral ulcers, alopecia
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Term
How do you diagnose systemic lupus erythematosus (SLE) |
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Definition
- + anti-nuclear ab (ANA) - best initial test, not specific + RF
- + anti-double stranded DNA & Anti-smith Ab 100% spp for SLE (not sensitive)
- antiphospholipid ab syndrome (APLS): increased risk of arterial & venos thrombosis
- + anticardiolipid Ab assoc w/ false + VDRL/RPR
- lupus anticoagulant
- B-2 glycoprotein I ab. women w/ APLS may have frequent miscarriages, livedo reticularis
4. CBC: +- hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia |
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Term
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Definition
sun protection
hydroxychloroquine (for skin lesions)
NSAIDs or acetaminophen for arthritis
+- pulse dose steroids
cytotoxic drugs (methotrexate, cyclophosphamide) |
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Term
systemic connective tissue d/o : thickened skin (sclerodactyl), lung, heart, kidney, and GI tract |
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Definition
scleroderma (systemic sclerosis) |
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Term
tight, shiny, thickened skin (localized or generalized) d/t fibrous collagen buildup |
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Definition
scleroderma (systemic sclerosis) |
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Term
Clinical manifestations of scleroderma (systemic sclerosis) |
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Definition
- Limited cutaneous systemic sclerosis "crest syndrome"
- Calcinosis cutis
- Raynaud's phenomenon
- Esophageal motility d/o
- Sclerodactyl (claw hand)
- Telangectasia
- MC type
- affects the face, neck as well as distal to elbows & knees
- Raynauds phenomenon : worse w/ cold, smoking, or emotional stress. CCB tx of choice
2. Diffuse cutaneous systemic sclerosis: skin thickening - trunk & proximal extremities |
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Term
How do you diagnose scleroderma (systemic sclerosis) |
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Definition
- + anti-centromere Ab: assoc limited/CREST dz - more specific. Better prognosis
- + anti-scl-70 Ab: assoc w/ diffuse dz & multiple organ involvement. + ANA (nonspp)
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Term
Treatment of scleroderma (systemic sclerosis) |
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Definition
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Term
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Definition
vasodilators (CCBs, prostacyclin) |
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Term
autoimmune d/o attacking exocrine glands.
salivary glands --> xerostoma (dry mouth); lacrimal glands --> dry eyes (keratoconjunctivitis sicca) & parotid enlargement. Thyroid gland dysfxn |
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Definition
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Term
Primary sjogren's syndrome |
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Definition
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Term
secondary sjrogens syndrome |
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Definition
assoc w/ other autoimmune disorders - SLE, RA, ect |
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Term
Activation of the immune system possibly triggered by viruses aggregation of lymphocytes (ex CD4 cells) in exocrine glands (lymphocytic invasion).
HLA-DR52 seen in 85% of patients |
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Definition
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Term
increased incidence of lymphoma, interstitial nephritis, and pneumonia |
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Definition
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Term
How do you diagnose sjrogen's syndrome? |
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Definition
ANA: esp antiSS-A (Ro) & antiSS-B (la)
+RF
Shirmer test (decreased tear production) |
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Term
How do you treat sjrogen's syndrome? |
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Definition
- artificial tears, pilocarpine (cholinergic) for xerostoma
- Pilocarpine: cholinergic drug that increases lacrimation & salivation (SLUDD-C) SE: diaphoresis, flushing, sweating, bradycardia, diarhea, N/V, incontinence, blurred vision. Cyclosporine may help w ocular symptoms
- Cevimeline (Evoxac): stimulates muscarinic cholinergic receptors
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Term
widespread muscular pain (us chronic), fatigue, muscle tenderness, HA's, poor sleep/memory problems (may be d/t increase in pain perception, increased substance P) |
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Definition
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Term
MC in middle aged women. increased incidence w/ RA, SLE, ankylosing spondylitis |
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Definition
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Term
Clinical manifestations of fibromyalgia |
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Definition
diffuse pain (esp in the morning)
extreme fatigue
stiffness
painful, tender, joints
sleep disturbances
sx often worsened w/ physical/psych stress |
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Term
How do you diagnose fibromyalgia? |
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Definition
- diffuse pain in 11 out of 18 trigger points > 3 mo + widespread pain
- Biopsy: "moth-eaten" appearance type 1 muscle fibers, injury to muscle.
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Term
how do you treat fibromyalgia? |
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Definition
TCA's, cymbalta, SSRI's, neurontin, exercise
Pregabalin only drug FDA approved
swimming preferred exercise (relaxing effect of water on muscles) |
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Term
idiopathic inflammatory condition causing synovitis, bursitis, & tenosynovitis --> aching/stiffness of proximal joints (shoulder, hick, neck) in pts > 50 |
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Definition
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Term
________ is closely related to giant cell arteritis |
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Definition
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Term
Clinical manifestations of polymyalgia rheumatica |
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Definition
- bilateral prox jt pain/STIFFNESS morning stiffness > 30 mins of the pelvic and shoulder girdle
- difficulty w/ combing hair, putting on coat, getting out of chair
- no severe muscle weakness
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Term
How do you diagnose polymyalgia rheumatica |
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Definition
clinical dz
increased ESR, anemia (normochromic normocytic)
+- plts (acute phase reactant) |
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Term
how do you treat polymyalgia rheumatica |
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Definition
corticosteroids (low dose - 10-20 mg/d); methotrexate |
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Term
idiopathic inflammatory muscle disease of proximal limbs, neck, pharynx |
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Definition
polymyositis & dermatomyositis |
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Term
Clinical manifestations of polymyositis & dermatomyositis |
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Definition
- progressive symmetrical proximal m. weakness (usually painless)
- dysphagia
- skin rash
- polyarthralgias
- muscle atrophy (difficulty rising from chair/climbing stairs, combing hair, problems w/ overhead movement)
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Term
Diagnosing polymyositis & dermatomyositis |
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Definition
- increased muscle enzymes: increased aldolase, creatine kinase ); increased ESR, + muscle bx, abnormal EKG
- + anti-Jo 1 Ab: myositis specific antibody. assoc w/ interstitial lung fibrosis, "mechanic hands" (hyperkeratotic "cracked hands" with dirty appearance
- + anti-SRP Ab; (signal recognition particle ab). almost exclusively seen w/ PM. Assoc w/ more prominent muscle weakness/atrophy & cardiac manifestations
- + anti-Mi-2 Ab: specific for dermatomyositis
- muscle bx: endomysial invovlement w/ PM; perifascicular/perivascular involvement w/ DM
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Term
Dermatomyositis: pathognomonic |
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Definition
- heliotrope (blue-purple) upper eyelid discoloration
- gottron's papules: raised violaceous scaly eruptions on the knuckles. malar rash w/ erythema (including nasolabial folds) photosensitive poikiloderma "shawl or V sign," diffuse alopecia
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Term
How do you diagnose polymyositis & dermatomyositis? |
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Definition
muscle enzymes: increased aldolase, creatine kinase
increased ESR
increased incidence of malifnancy |
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Term
how do you treat polymyositis & dermatomyositis? |
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Definition
corticosteroids 1st line tx
+- methotrexate, azathioprine, IV immune globulin |
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Term
Gout mostly happens in.... |
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Definition
mostly adult men (& postmenopausal women)
middle aged |
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Term
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Definition
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Term
What does gout show in the synovial fluid? |
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Definition
negatively birefringent: needle-shaped |
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Term
Most frequently affected joints for gout |
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Definition
- first MTP joint MC (podagra) 50% initially, 90% eventually
- 80% monoarticular typically lower extremities (ankle, knees, foot)
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Term
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Definition
"mouse bite" (punched out) erosions |
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Term
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Definition
- acute attacks - NSAIDs first line, colchicine, steroids
- chronic - urate lower agents (allopurinol, febuxostate, probenacid) colchicine can also be used .
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Term
Pseudo gout usually happens in... |
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Definition
female predominance, elderly
less common than gout |
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Term
calcium pyrophosphate dihydrate |
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Definition
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Term
What does synovial fluid show in pts with pseudo gout? |
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Definition
calcium pyrophosphate dihydrate |
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Term
What joints are most frequently affected in pseudogout |
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Definition
knees, wrists, MCP joints, elbows, MTP |
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Term
x-ray findings in pseudogout |
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Definition
chondrocalcinosis- calcification of the cartilage |
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Term
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Definition
- acute attacks: steroids 1st line (intraarticular), NSAIDs, colchicine
- Chronic managmement: NSAIDS, +- colchicine
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Term
Important to remember during gout and pseudogout |
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Definition
Note that chronic management NOT initiated during acute attack (may precipitate an attack)
- any acute increase or decrease in uric acid levels may precipitate an attack moreso than the actual uric acid levels |
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Term
systemic vasculitis of medium/small arteries --> necrotizing inflammatory lesions |
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Definition
polyarteritis nodosa (PAN) |
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Term
polyarteritis nodosa (PAN) has increased association with... |
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Definition
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Term
increased micro aneurysms w/ aneurysmal rupture --> hemorrhage, thrombosis--> organ ischemia or infarction
muscular arteries involved
Type III HSN |
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Definition
polyarteritis nodosa (PAN) |
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Term
Clinical manifestations of polyarteritis nodosa (PAN) |
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Definition
- renal: HTN (d/t increased renin), renal failure. usually no glomerulonephritis. No capillaries involved
- constitutional: fevers, myalgias, arthritis. lungs usually spared
- CNS: neuropathy, amaurosis fugax, peripheral neuropathy, mononeuritis complex
- Dermatologic: livedo reticularis, purpura, ulcers, gangrene, nodules, Raynauds phenomenon
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Term
How do you diagnose polyarteritis nodosa (PAN) |
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Definition
- increased ESR; angiography
- MC seen in men ~ 45 yo
- classic PAN is ANCA negative
- BX: necrotizing inflammatory lesions. no granulomas
- Renal or mesenteric angiography: microaneurysms c/ abrupt cut-off of small arteries
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Term
how do you treat polyarteritis nodosa (PAN) |
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Definition
steroids (+- cyclophosphomaide if refractory)
+- plasmapharesis in pts w/ HBV |
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Term
Primary joints affected with RA |
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Definition
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Term
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Definition
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Term
soft, warm, BOGGY, tender joints |
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Definition
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Term
Worse after resting. morning stiffness that lasts @ least or > 30 minutes |
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Definition
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Term
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Definition
osteopenia
symmetric joint narrowing |
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Term
laboratory findings positive for RA |
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Definition
Positive RF
anti CCP ab
ESR, CRP |
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Term
chronic inflammatory dz w/ persistent symmetric polyarthritis w. bone erosions, cartilage destruction, and joint structure so (d/t destruction by pannus) T cell mediated |
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Definition
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Term
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Definition
rare d/o triad
RA+splenomegaly+decreased WBC/repeated infections |
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Term
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Definition
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Term
Which test is the best initial test for RA, sensitive but NOT specific |
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Definition
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Term
which test is the most specific for RA? |
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Definition
Anti-citrullinated peptide antibodies (anti-CCP) |
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Term
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Definition
arthritis >= 3 joints
morning stiffness
disease duration > 6 weeks |
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Term
Radiologic findings: narrowed joint space (osteopenia/erosions), subluxation, deformities |
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Definition
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Term
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Definition
PROMPT initiation of DMARDs (methotrexate, hydroxychloroquine, leflunamide, minocycline azathioprine) to reduce permanent damage to the joint, cartilage, bone
NSAID for pain control, low dose steroids |
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Term
autoimmune response to infection in another part of the body |
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Definition
reactive arthritis (Reiter's syndrome) |
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Term
Reactive Arthritis (Reiters syndrome) |
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Definition
- asymmetric inflammatory arthritis
- conjunctivitis/uveitis
- urethritis, cervicitis
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Term
Reactive Arthritis (Reiters syndrome) is MC seen with... |
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Definition
chlamydia
+- seen with gonorrhea
GI (salmonella, shigella, campylobacter, yersinia) |
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Term
Clinical manifestations of Reactive Arthritis (Reiters syndrome) |
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Definition
conjunctivitis
urethritis
arthritis (esp. lower extremities)
sausage toes/fingers
keratoderma blennorrhagica (hyperkeratotic lesions on palms/soles)
circinate balantitis |
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Term
how do you diagnose reactive arthritis (reiters syndrome) |
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Definition
+ HLA-B27 (80%)
CBC: increased WBC (10,000-20,000)
increased ESR
normochromic anemia
increased IgG
synovial fluid: WBC 1,000-8,000 cells/mm3
synvoial fluid bacterial culture negative (aseptic) |
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Term
how do you treat reactive arthritis (reiters syndrome) |
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Definition
NSAIDS mainstay of therapy
abx to treat infx that triggered disease
if no repsonse to methotrexate --> sulfasalazine, steroids.
anti-TNF agents (etanercept, infliximab) |
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