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Flashcards in week 7- MSk 2 Deck (67)
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• SLE:

o chronic inflam dz
o type III hypersensitivity
o potential type II involvement → auto-Abs against nuclear components of own cells


• SLE epidem:

o F > M 15:1 in child-bearing age
o black 3x > white
o Any age, onset usu 16-55


• SLE etio:

o Genetic:1st-deg relatives 8-9x ↑risk; Epigenetic hypomethylation
o Hormones: E2 (OCPs ↑ risk 50%), P, T, DHEA, PRL, modulate incidence and severity
o Environ: trigger or exacerbate: Infx (Mycobacteria, trypanosomiasis, EBV), Silica dust, Vaccines (HBV, HPV), Allergies (meds, abx), UV light, Stress, Surgery, Pg
o Drug rxn (drug-induced lupus): Reversible, usu dt tx long-term illness, mimics systemic lupus, Statins, Hydralazine, Methyldopa, chloropromazine


• SLE ssx:

o Mild  moderate  fulminant
o Usu Fatigue, fever,↓wt
o Mucocutaneous: > 90%, non-painful oral ulcers, nail fold infarcts, periungular erythema, spinter hemorrages, Raynauds, alopecia, malar “butterfly” rash (30%)
o MSk: often 1st sx, symmetric, non-deforming arthritis of digits, wrists, knees, MTP, hard to distinguish from RA if other sx absent, prolonged AM stiffness (50%), nodules (7%), myalgia
o Renal: most, only 50% clinical, proteinuria, micro hematuria, GN → ESRF, death
o CVS: mc pericarditis  chest pain, mimic MI, Libman-Sacks endocarditis (sterile), atherosclerosis → angina, MI
o Resp: > 50%; mc pleurisy, pleural effusion, pneumonitis, interstitial lung dz, pulm HTN, alveolar hemorrhage; Pulm emboli if lupus anti-coag present
o GI: N/V/D, reflux, non-specific abd pn, peptic ulcers, vasculitis→pancreatitis, peritonitis, colitis
o CNS: ↓cognition, organic brain dos, delirium, psychosis, szs, HA, peripheral neuropathies
o Blood and lymphatics: anemia, thrombocytopenia, leukopenia, peripheral LA, SM, a-PL & a-cardiolipin ↑thrombosis
o Ophthalmologic: keratoconjunctivitis sicca common, Cotton wool exudates dt retinal vasculitis, episcleritis, scleritis, anterior uveitis lc


• SLE labs:

o ANA 95-100%, a-dsDNA (60%, active dz), ↓serum C3, C4 (60%, w flare), + RF (20%)
o Leukopenia, lymphopenia, thrombocytopenia. Abn urinary sediment. Proteinuria. ↑PT, Hypoalbuminuria, ↑creatinine , (+) Coombs, Immune complex assay (cryoglobulins, Raji cell test, C1q precipitins).
o Auto-Abs: a-histone (drug induced, other AI), Anti-ENA- Sm, RNP, RNA-protein complexes (↑spec for SLE, MCTD), Anti-SSA(Ro), SSB(La) (Sjogrens)


• SLE imaging:

o MRI/Cerebral angiography in CNS lupus
o CXR for pulm infiltration, pleural effusion
o Echo for pericardial effusion


• SLE dx criteria:

o 4+/11: (nothing is pathognominc)
o 1. Malar rash,
o 2. Discoid lupus
o 3. Potosensitivity (UV)
o 4. Oral or nasopharyngeal ulcers
o 5. Non-erosive arthritis
o 6. Renal do: proteinuria (>3+ dipstick or >0.5 g/d ) or cylindruria (cellular casts)
o 7. Neuro do: Szs or psychosis
o 8. Serositis: pleuritic, pericarditis, peritonitis
o 9. Bood do: Hemolytic or leucopenia (↓4,000/mL), lymphopenia ( ↓1500/ul ), thrombocytopenia (↓100,000/uL) w/o offending drug
o 10. ANA+ (95-100%) w/o drugs
o 11. Immune do: (+) anti-Sm, anti-ds DNA, anti-PL, or false (+) sero test for syphilis


• SLE ddx:

o RA, MCTD, systemic vasculitis, scleroderma
o metastatic malignancy, fever of unknown origin
o inflam myopathies, viral hepatitis, sarcoidosis
o acute drug rxn, drug induced rxns
o psychogenic rheumatism
o many cutaneous rashes


• progressive systemic sclerosis (scleroderma)

o rare, chronic dz w immune activation, vascular damage,↑ECM, ↑collagen
o localized or systemic →limited cutaneous or diffuse
o CREST syndrome


• scleroderma etio:

o Molecular mimicry w/ CMV
o Contaminated rapeseed oil
o Polyvinyl chloride
o Epoxy resins
o Aromatic hydrocarbons
o Similar to graft vs host dz


• Localized Scleroderma:

o Morphea
o Linear scleroderma (line of thick skin on extremities)
o Scleroderma en coup de sabre (forehead and face)
o No internal or organ involvement


• Systemic Sclerosis- Limited cutaneous:

o Skin thick distal to elbow or knee
o Can have face involvement
o Begins w raynauds w gradual onset heartburn, tender digital pitting scars or ulcers, thick skin over fingers
o Later pulm fibrosis and pulm HTN


• Systemic Diffuse Scleroderma:

o Any skin affected
o Fibrosis of skin, pulmonary parenchymal and GI tract
o Small Vessel Vasculopathy: raynauds, renal crisis, pulm artery HTN
o More rapid, skin changes quickly, raynauds, internal organ involvement in 2 yrs
o Fibrosis of lungs, heart, GI, renal crisis (↓ blood flow dt vasculitis)


• CREST syndrome

o Mild variant of scleroderma
o slow onset and progression, skin hardening usu confined to hands and face, internal organ involvement less severe
o much better px
o Calcinosis
o Raynaud's phenomena
o Esophageal dysfunction
o Sclerodactyly
o Telangiectasia


• Scleroderma ssx:

o Onset: Fatigue, Swollen digits, stiff jts, weak, pain, insomnia, skin discoloration
o Skin: induration symmetric, mb only fingers (sclerodactyly), distal arms or all body; as progresses, skin becomes taut, shiny, hyperpigmented, face becomes mask like; telangiectasias on fingers, chest, face, lips, tongue; subQ calcifications usu fingertips
o MSk: jt contractures, tendon friction rubs, myopathy, myositis, cutaneous calcifications, synovitis, neuropathies
o GI: esophageal dysfxn in most. dysphagia, acid reflux (mb severe), bloating, ↓wt, constipation, gastroparesis, SIBO, Barrett’s metaplasia in 1/3m ↑risk adenocarcinoma
o CV: arrhythmia, EKG abn; pericarditis
o Resp: leading cause of death; Dry cough, DOE, pulm HTN
o Kidney: mb malig HTN


• Scleroderma dx criteria:

o *may miss ppl in early dz w CREST
o Major: skin thick just proximal to MCP’s
o Minor: Sclerodactyly, Permanent ischemia of fingertips, Bibasilar pulm fibrosis


• Scleroderma labs:

o ANA: almost all (+)
o ANA w centromere pattern usu have better course, but more pulm fibrosis
o Anti-Scl 70: ↑risk pulm fibrosis
o CMP: renal involvement


• Scleroderma imaging:

o Echo: PAH
o CT: pulm fibrosis


• Polymyositis/Dermatomyositis:

o Idiopathic inflam myopathies
o present clinically similar, but have distinct immunopathogenicities
o PM: direct T cell mediated muscle injury, in fascicle
o DM: immune complex deposition in blood vessels (B cell)
o DM:↑risk malig (adenocarcinomas of lung, cx, ovaries, pancreas, bladder, stomach)
o 1/100,000
o Peak 40, or any age
o F:M 2:1


• PM/DM ssx:

o symmetric proximal mm weakness
o mm pain (50%)
o Fever, ↓wt, Raynaus, nonerosive inflam polyarthritis
o Rare: cardiomyopathy, dysphagia via weak esophagus, interstitial lung dz (mb fatal)
o DM presents w characteristic rashes
o Gottron's papules, shawl sign, heliotrope peri orbital rash, erythroderma


• PM/DM work-up:

o PE: Gowers Test (+)
o CK, LDH aldolase, AST, ALT
o Anti-Jo-1, Anti-SRP, Anti-Mi-2 (+ 30%)
o Muscle bx
o ANA often (+). If anti-SSA, SSB (+) consider overlap d/o
o skin bx (DM)
o MRI: to monitor tx


• PM/DM ddx:

o ALS, Myasthenia gravis, inclusion body myositis
o muscular dystrophies
o Hypothyroid
o HIV, acute infx
o meds (steroids, statins)


• polymyalgia rheumatic:

o severe aching and stiffness in neck, shoulder & pelvic girdles
o =rheumatic dz, but unk etio
o Severe pain & stiffness in proximal mm w/o permanent weakness or atrophy
o No evidence on bx.
o Mm strength and EMG normal
o >50, ↑incidence w age, 1/200
o F:M 2:1


• PMR ssx:

o mb acute or subacute
o Pain, stiffness symmetric in neck, shoulder, pelvic girdle
o ↓ROM of joints 2nd to pain
o marked morning stiffness, difficult to get out of bed
o Subjective weakness
o Tenosynovitis
o Malaise, fatigue, depression, anorexia, ↓wt, fever
o Palpable, mild, synovitis in knees, wrists, MCP jts


• PMR labs:

o ESR usu 40-100mm/h
o CRP >5 mg/L
o Normocytic anemia
o ↑ plts
o Mb ↑transaminases
o ANA, RF, CCP (-)


• PMR imaging:

o Plain films normal
o MRI may reveal tenosynovitis, bursitis


• PMR dx criteria:

o >50 at onset
o BL aching morning stiffness (last 30+ mins), at least 1 mo
o stiffness in 2+/3 area: neck or torso, shoulders or proximal arms, hips or proximal thighs
o ESR ≥40 mm/h


• Concomitant of PMR:

o tendency to Temporal Arteritis (Giant Cell Arteritis)
o Chronic vasculitis of large and medium vessels


• Temporal arteritis ssx:

o Localized HA of new onset
o General ill feeling
o Tender or ↓pulse of affected artery
o Jaw or tongue claudication
o Sudden, painless blindness


• Temporal arteritis PE:

o Temporal or occipital artery tenderness
o ↓ temporal artery pulsations
o Red, nodular swollen scalp arteries (rare)
o Ill appearing pt