Rheumatology Flashcards
SLE
autoimmune disorder of connective tissues
- mc F (9:1), onset 20-40s, AA, Hisp, Native Amer
- rf: genetics, sun exposure, infx, estrogen,
- drug-induced (procainamide, hydralazine, INH, quinidine) + ANTI-HISTONE AB
- sx-
- Triad: JOINT PAIN (90%), FEVER, MALAR “BUTTERFLY RASH” (spares nasolabial folds)
- serositis (pericardidits, pleuritis)
- Discoid Lupus - annular, red patches on face, scalp, heals w/ scarring
- Systemic - CNS, cardiovascular, glomerulonephritis, retinitis, oral ulcers, alopecia
-dx-
+ ANA (initial best test, not specific) + RF
+ ANTI DOUBLE-STRANDED DNA + ANTI-SMITH AB 100% specific for SLE, not sensitive
- ANTIPHOSPHOLIPID AB SYNDROME (APLS): inc risk for arterial + venous thrombosis - may have freq miscarriage
-CBC +/- anemia, leukopenia, lymphopenia, thrombocytopenia, assoc w/ HLA-DR
-tx-
-skin: sun protection, hydroxychloroquine for lesions
-arthritis: NSAIDs or tylenol
+/-pulse-dose corticosteroids
-cytotoxic drugs (methotrexate, cyclophosphamide)
scleroderma (systemic sclerosis)
systemic connective tissue disorder (thickened skin - sclerodactyly), lung, heart, kidney + GI tract
-sx- tight, shiny, thick skin due to fibrous collagen buildup
- MC type: LIMITED cutaneous systemic sclerosis “CREST SYND”: Calcinosis cutis, Reynaud’s phenom, Esophageal motility disorder, Sclerodactyly (claw hand), Telangiectasia
- affects face, neck, distal to elbows/knees, spares trunk
-DIFFUSE cutaneous systemic sclerosis: skin thickening - trunk and proximal extremities
-dx-
+ ANTI-CENTROMERE AB - assoc w/ limited/CREST, more specific, better prognosis
+ ANTI-SCL-70 AB - assoc w/ DIFFUSE dz + multiple organ involvement
-tx- DMARDS, cortico
sjogren’s
autoimmune, attacks exocrine glands (salivary –> xerostomia, lacrimal glands –> keratoconjunctivitis sicca, parotid gland enlargement)
-thyroid dysfx common
- primary SS - occurs alone
- secondary SS - assoc w/ other autoimmune
- HLA-DR52 seen in 85%
- inc incidence of non-hodgkin lymphoma, interstitial nephritis, pneumonitis
-dx-
+ANA, esp antiSS-A (Ro) & antiSS-B (La)
+RF, +Schirmer test (dec tear production)
- tx-
- pilocarpine - cholinergic, inc lacrimation and salivation (SE diaphoresis, flush, sweat, bradycardia, D/N/V, incontinence, blurred vision)
- cevimeline (exovac) - stim muscarinic cholinergic receptors
polymyalgia rheumatica
idiopathic inflam condition causing synovitis, bursitis + tenosynovitis –> pain/stiffness of the proximal joints (shoulder, hip, neck) in pt >50yo
-closely related to GIANT CELL ARTERITIS
-sx- bilat proximal joint ache/STIFFNESS
morning stiffness > 30 min of pelvic, neck, shoulder, no muscle weakness
-dx- clinical dx
inc ESR, anemia, +/- inc platelets (acute phase rx)
-tx- low dose corticosteroids
NSAIDs, methotrexate
polymyositis + dermatomyositis
idiopathic inflam muscle dz of proximal limbs, ncec, pharynx; may affect heart, lungs & GI
- sx- PROGRESSIVE SYMMETRICAL PROXIMAL MUSCLE WEAKNESS (usually painless)
- dysphagia, skin rash, polyarthralgias, muscle atrophy
-dx-
-labs: inc muscle enzymes (inc aldolase, creatine kinase), inc ESR
+ANTI-JO 1 AB (myositis-specific antibody) - assoc w/ “mechanic hands”, GOTTRON’S PAPULES - raised violaceous scaly knuckle eruptions
+ANTI-SRP AB (signal recognition particle ab) - almost exclusively seen with PM
+ANTI-MI-2-AB (specific for dermatomyositis)
-muscle biopsy
-tx- high-dose cortico 1st line
methotrexate, IV immunoglobulin
gout
uric acid deposition in soft tissue, joints, bone - mc due to under-excretion
-mc common podagra (big toe)
-sx- attacks begin fast (max in 8-12 hrs), w/out tx can move polyarticular, more proximal and last longer –> devo into chronic arthritis
- dx-
- arthrocentesis –> NEG BIFERINGENT NEEDLE-SHAPED URATE CRYSTALS
- radiographs –> MOUSE/RAT BITE punched-out erosions
- clinical –> inc ESR + WBC in acute (serum urate levels don’t reflect joint involvement)
-tx-
NSAIDs, AVOID ASA (inc serum uric acid)
COLCHICINE
prophylaxis –> allopurinol, uloric
-complications: degenerative arthritis, 2ry infx, uric acid nephropathy, renal stones (inc solubility of Ca too), nerve impingement, frx
pseudogout (calcium pyrophosphate dihydrate deposition disease)
CALCIUM PYROPHOSPHATE crystals deposit in joints/soft tissue
-mc elderly women
- sx- acute arthritis (red, swollen, tender), KNEE MC
- CHONDROCALCINOSIS (cartilage calcification) - linear radiodensities seen on xray
- large joints - knee, wrist, elbow, angle,
-dx- arthrocentesis –> POSITIVELY BIREFRINGENT, RHOMBOID SHAPED CPPD CRYSTALS
- tx- INTRAARTICULAR CORTICO 1ST LINE
- NSAIDs, colchicine (acute or prophylaxis)
rhabdomyolysis
acute breakdown and necrosis of skeletal muscle
- MC assoc w/ immobility (fallen and can’t get up), crush injuries, overexertion, seizures, burns
- MEDS: STATINS, niacin, fibrates, cp450 inhibitors (macrolides, verapamil, diltiazem)
- dx-
- labs: inc CPK >20K, inc LDH, ALT
- HYPERKALEMIA (intracell K released from damage)
- HYPOCALCEMIA (bonds to damaged muscle)
- UA: dark urine + for heme but negative for blood (bc myglobinuria)
- tx-
- IV saline, mannitol to induce osmotic diuresis, bicarb to alkalinize urine
- calcium gluconate if hyperK w/ ECG findings
-complications: AKI (acute tubular necrosis) bc of excess myoglobin
juvenile idiopathic arthritis (rheumatoid)
autoimmune mono or polyarthritis in children <16yo (often resolves after puberty)
PAUCI-ARTICULAR (oligoarticular) 50%
- less than 5 joints involved in 1st 6 mo
- Type 1 may be assoc w/ IRIDOCYCLITIS (ANT UVEITIS)
- Type 2 assoc w/ increased incidence of akylosing spond
SYSTEMIC/ACUTE FEBRILE (STILL’S DZ) 20%
- daily arthritis, DIURNAL HIGH FEVER w/in 1st 6 mo
- SALMON-COLORED / PINK MIGRATORY RASH +/- koebner phenom
- assoc w/ hepatosplenomegaly, hepatitis, lymphadenopathy & serositis
POLYARTICULAR 30%
- ARTHRITIS > 5 SMALL JT in 1st 6 mo (usually symmetric); most similar to adult RA
- inc risk of IRIDOCYCLITIS
- RF neg assoc w/ better prognosis than RF pos
- dx- clinical
- labs: inc ESR, CRP, +ANA in pauci-articular, +RF in 15%
- tx- NSAIDs + or cortico
- methotrexate or leflunomide
- freq eye exams to detice iridocyclitis
osteoarthritis (OA)
articular cartilage damage + degeneration
- RF: obesity
- MC in wt-bearing joints, OA of hands MC in women
- narrow jt space, sclerosis + OSTEOPHYTE FORMATION
- sx-
- EVENING STIFFNESS -worsens throughout day + w/ change in weather, dec ROM, crepitus, absence of inflam signs
- HARD BONY JOINTS = osteophytes
- HEBERDEN’S NODE (palpable osteophytes @ DIP jt)
- BOUCHARD’S NODE - PIP osteophytes
-dx- XRAY - joint space loss, osteophytes
- tx-
- tylenol preferred intial tx in elderly w/ bleed risk + mild/mod dz
- NSAIDs more effective, cortico injections, sodium hyaluronate, glucosamine, chondroitin
rheumatoid arthritis
chronic inflam dz w/ persistent symmetric polyarthritis, bone erosion, cartilage destruction + joint structure loss
- T-cell mediated
- PANNUS - granulation tissue that erodes into cartilage + bone
- RF: female, smoking
- sx-
- prodrome: constitutional systemic sx like F, fatigue, WL, anorexia, dec ROM
- SMALL JOINT STIFFNESS - MCP, wrist, PIP, knee, MTP, shoulder, ankle
- MORNING JOINT STIFFNESS > 60 MIN after initiating MVMT (improves later in the day)
- SYMMETRIC ARTHRITIS - swollen, tender, red, boggy joint
- boutonniere deformity, swan neck deformity, ULNAR DEVIATION at MCP jt
-dx-
+RF best initial (sensitive, not specific), inc CRP, ESR
+ANTI-CYCLIC CITRULLINATED PEPTIDE AB - most specific for RA
-arthritis >3 joints, morning stiffness, disease >6 wks, anemia
-xray: narrowed joint space (osteopenia/erosions), ulnar deviation
- tx-
- DMARDs - promptly reduces permanent damage
- METHOTREXATE 1st LINE
- NSAIDs first line for pain control; CORTICO 2nd line if no relief w/ nsaids
giant cell (temporal) arteritis
systemic, granulomatous (cell-mediated), chronic vasculitis (inflam of large/med arteries) mostly affecting cranial arteries (head + neck)
- same clinical spectrum as POLYMYALGIA RHEUMATICA (present in 50% of cases)
- MC women >50y
- etiology:
- idiopathic, +/- autoimmune
- vasculitis of extracranial branches of carotid artery: temporal, occipital, ophthalmic and posterior ciliary
- sx- HEADACHE MC (new onset, local, unilateral, temporal and lancinating), JAW CLAUDICATION w/ mastication, ACUTE VISION DISTURBANCES (amaurosis fugax)
- constitutional sx, thickened temporal artery (tender), AORTIC ANEURYSM (may have TIA/CVA sx)
- blindness MC complication
- dx- clincial
- increased ESR (>100), inc CRP, normo anemia
- temporal artery bx (may have skip lesions so negative bx is not for sure) –> bx: MONONUCLEAR LYMPHOCYTE INFLITRATION, MULTINUCLEATED GIANT CELLS, LAMINA CELL DEGRADATION
- tx- HIGH DOSE CORTICO (40-60 mg/d x6 wks)
- w/ high suspision, start predinisone before bx confirms (improve 24-72 hrs)
- steroid-sparing anti-inflam (methotrexate, azathioprine) if cortico refractory
behcet’s syndrome
multisystemic autoimmune disorder char by RECURRENT, PAINFUL ORAL AND GENITAL ULCERS (aphthous), erythema nodosum, eye (uveitis, conjunctivitis), arthritis + CNS involvement
-tx- corticosteroids w/ flares
takayasu’s arteritis
chronic large-vessel arteritis affects AORTA, AORTIC ARCH + PULMONARY ARTERIES
- women 80-90%, younger onset 10-40y, ASIANS
- cell-mediated vascular infiltration of all layers of large arteries (inflam, granuloma formation, giant cell, killer T, lymphocyte)
- sx-
- systemic phase (early): fatigue, arthralgias, myalgias, WL, night sweats, low grade fever
- vascular occlusive phase (late): vessel stenosis/occlusion/ischemia, CORONARY ARTERY MI, common carotid (TIA, CVA, HA, vision change, syncope), renal artery (HTN), subclavian (claud), posterial vertbral (visual change, dizzy), pulmonary artery (dyspnea, hemoptysis), LOWER EXTREMITY CLAUDICATION
- ARTERIAL BRUITS, DIMINISHED PULSES, ASYMMETRIC PB >10mmHg
- dx-
- ANGIOGRAPHY to confirm + determine extent
- helical CT angio or MRA may show thick/edema arteries
- inc ESR/CRP, normo anemia
- tx-
- HIGH DOSE CORTICO - 60mg/d x 6wks w/ taper
- cytotoxic (chronic dz): methotrexate & azathioprine
kawasaki syndrome (mucocutaneous lymph node syndrome)
?? unidentified resp agent or virus w/ propensity for vascular tissue
- mc kids <5y, boys, asians
- medium + small vessel necrotizing vasculitis incluing CORONARY ARTERIES
- sx- “warm + CREAM” = fever + 4 of 5:
- Conjunctivitis - bilat and nonexudative
- Rash - polymorphous (erythem or morbiliform or macular)
- Extremity (peripheral) changes - desquamation, edema, erythema of palms/soles, beau’s lines, arthritis
- Adenopathy - cervical lymph (unilat)
- Mucous membrane - pharyngeal erthema, lip swelling/fissures, strawberry tongue
-complications: coronary vessel arteritis; CORNONARY ARTERY ANEURYSM, MYOCARDIAL INFARCTION
- dx-
- labs: inc ESR/CRP, leukocytosis, rx thrombocytosis (inc platelets, normo/normo anemia, STERILE PYURIA, may need echo/angio
-tx- IV IMMUNE GLOBULIN + HIGH DOSE ASPIRIN
