Autoimmune Path

Front 1

bla-b27 assoc

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**as, post gc arthritis, acute ant uveitis

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dr4

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ra, t1dm

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dr3

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chronic active hepatitis, primary ss, t1dm

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sle pathogenesis

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fx9>m, black women

anti dsdna (ana w/ if, histones, non-histone pro, nucleolar, patterns homogensou, periph, nucleolar, speckled most common, sensitive but not specific), anti sm, anti pl (cardiolipin), lupus anticoag (pro-coag, miscarraige, ischemia), fals pos syphillis

genetic (fh, relatives auto ab, 34% mz concordance), drugs (hydralazine, procainamide, d-penicillamine, inh) --> ana, also uv, viruses, sex hormones

b-cell hyperact --> self-ab --> icd/t3hs for visceral lesions (bv, kidney, ct, skin, acute necrotizing vasculitis - sm aa, fibrinoid dep, chronic --> fibrous thickening, narrow lumen), t2hs for rbc/wbc/platelet

le cell: hematoxylin body, formed when pmn/macrophage engulf nuc of inj cell, ana bind, c' fix, test by lyse pmn, add pt serum

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sle criteria

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malar rash: butterfly, 50%, also extremities/trunk, liquefactive degen basal layer, edema at d-e jct, perivascular mononuc infil, fibrinoid vasculitis, ig/c' dep on d-e jct w/ if
discoid rash: rarely systemic (5-10% after yrs), skin plaques (edema, erythema, scaliness, follicle plugging, atrophy w/ erythematous border)
photosensitivity
oral ulcers
arthritis: 2+ periph jts, tenderness/swelling/effusion, non-erosive synovitis, little deform, no pannus, pmn/fibrin in synovium
serositis: pleuritis (fibrinous exudate, later thickening/coating w/ fibrous tissue, edema, hemorrhage), pericarditis (myocarditis less common, resting tachy, non-bac verrucous endocarditis, libman-sacks, 1-3mm warty dep on valves, accel arthersclerosis)
renal d/o: persistant proteinuria, cell casts, class i (nl lm/em/if), ii (mesangial gn, mild hematuria, transient proteinuria), iii (focal prolif gn, <1/2 glomeruli, 1/3 proteinuria), iv (diffuse prolif gn, endo/epithelial cells - cresents - fibrinoid necrosis, hyaline thrombi, wire-loop lesions - thick capillaries - subendo dep em), v (membranous gn, severe proteinuria, subepi dep em)
neuro d/o: seizure/psychosis w/o drugs or metab d/o (uremia, ketoacidosis, electrolyte balance), non-inflam occlusion of sm vessels by intimal prolif (acute vasculitis due to anti pl ab dmg to endothelium)
heme d/o: hemolytic anemia w/ reticulocytosis, leukopenia, lymphopenia, thrombocytopenia w/o drugs, splenomegaly (capsular thickening, follicular hyperplasia, onion-skinning of penicillary aa), acute vasculitis in liver portal tracts w/ lymphocyte infiltrate
immuno d/o: anti dna, anti sm, igg/m anti cardiolipin ab, pos lupus anticoag, false pos t. pallidum sero w/ neg immobiliation/ftaa, ln hyperactive follicles/plasma cells
ana: abn titer w/o drugs

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sle sxs

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in dec order: heme, arthritis, skin, fever, fatigue, wl, renal, cns, pleuritis, myalgia, pericarditis, gi, raynaud, ocular, periph neuropathy

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ss

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immune dest lacrimal/salivary glands (primary sicca syndrome), keratoconjunctivitis sicca (dry eyes, blurring, burning, itching, thick sec), xerostomia (dry mouth mucosa, dysphagia, dec taste), if w/ other autoimmune dz (usu ra) then 2ndary, due to lymphoid infil (act cd4, b cell), 75% rf pos, 50-80% ana pos, 25% le pos, ***90% ssa/ssb (ro/la, rnp ag, also sle), high ssa titer --> extraglandular manifestation, assoc hla, ebv?, 40x lymphoma (b cell marginal zone type)

dx lip bx since usu spared from inf/trauma, look for perivascular/periductal inflam, lymphoid follicles w/ germinal centers, can look like hashimoto's

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mikulicz's syndr

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lacrimal/salivary gland enlargment due to sarcoid, leukemia, lymphoma, ss

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systemic sclerosis

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scleroderma, excess fibrosis throughout body, skin common, also gi (90%+, most severe esophagus, mm atrophy, replace collagen --> dysmotility, mucosa thin --> ulceration), kidney (2/3, intimal thickening, collagen dep interlobular aa, concentric prolif intimal cells), heart (pericarditis, myocardial fibrosis), msk (synovitis --> fibrosis, no jt destruction), lung (interstitial fibrosis, cyst-like cavities, thick small vessels), 3x f>m, peak 50-60, most severe black women, usu diffuse (visceral --> renal fail, card fail, pulm insuff, gi), skin drawn tight (knuckle erosion, thin epidermis, thick dermis)

immuno hypothesis: unknown ag --> t-cell act --> cytokines --> inc collagen synth by fibroblast

vascular hypothesis: endothelial inj (pdgf, fibroblast chemotaxis) --> platelet agg --> periadventitial fibrosis --> narrowed microvasculature --> ischemia, can see capillary dilation/destruct

very specific ab: anti-sc170 (anti-dna topoisomerase i, more likely pulm fibrosis, periph vascular dz), anticentromere (96% crest syndr)

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crest syndr

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localized scleroderma, milder, limited skin involvement, Calcinosis (ca dep in ct), Raynaud's, Esophageal dysmotility, Sclerodactyly (digits frozen in semi-flexion), Telangiectasia

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scleroderma

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systemic sclerosis, excess fibrosis throughout body, skin common, also gi (90%+, most severe esophagus, mm atrophy, replace collagen --> dysmotility, mucosa thin --> ulceration), kidney (2/3, intimal thickening, collagen dep interlobular aa, concentric prolif intimal cells), heart (pericarditis, myocardial fibrosis), msk (synovitis --> fibrosis, no jt destruction), lung (interstitial fibrosis, cyst-like cavities, thick small vessels), 3x f>m, peak 50-60, most severe black women, usu diffuse (visceral --> renal fail, card fail, pulm insuff, gi), skin drawn tight (knuckle erosion, thin epidermis, thick dermis)

immuno hypothesis: unknown ag --> t-cell act --> cytokines --> inc collagen synth by fibroblast

vascular hypothesis: endothelial inj (pdgf, fibroblast chemotaxis) --> platelet agg --> periadventitial fibrosis --> narrowed microvasculature --> ischemia, can see capillary dilation/destruct

very specific ab: anti-sc170 (anti-dna topoisomerase i, more likely pulm fibrosis, periph vascular dz), anticentromere (96% crest syndr)

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inflam myopathy

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ana ab (anti-jo-1, anti trna synthetase, 15-25%, coexist ipf)
dx: clinical, emg, enz, mm bx

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dermatomyositis

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adult/children, heliotrope skin rash (lilac, upper eyelid), periorbital edema, grotton's lesion (red erutpion on knuckle, elbow, knee), mm weakness (symmetric, prox first), capillary attack by ab/c' --> myocyte necrosis, women inc visceral cancer, children gi ulcer, soft tissue calcification

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polymyositis

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no cutaneous involve, adults, cd8 mediated, endomysial inflam w/o vascular inj

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incl body myositis

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distal mm first, asymmetric, 50+ yo, cd8 mediated, frozen section shows rimmed vacuoles

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mctd

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mixed ct dz, elements of sle, polymyositis, scleroderma, high titers u1 rnp, few renal dz, good response to steroid

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vasculitis

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pan (necrotizing inflam bv walls, non-inf)