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Scleroderma

Rheumatology

QuestionAnswer
Dx gold std for PM/DM bx (open bx preferred; can do needle)
PM/DM epi 2 peaks (kids; >50 yo); very rare; often assoc w/malig (esp DM) & other autoimm dz
DM/PM Pathophys: autoimmune dz freq assoc w/ other autoi dz; injury to mx fibers is immune-mediated; pos response to immunosupp tx; unique autoAbs
DM/PM Pathophys: environmental triggers UV light, infection, drugs
Polymyositis = CD8+ T cell-mediated antigen specific toxicity, class I MHC expression on mx cell fibers
Dermatomyositis = Perivascular B cells and CD4+ T cells, Ig and complement deposition in capillaries
Inclusion body myositis = CD8+ T cells, intracellular vacuoles
Rarest of the inflammatory myopathies = PM
Considered pathognomonic of dermatomyositis Gottron’s papules
Gottron’s sign: a macular rash in same areas, no papules
V sign: macular photosensitivity rash over anterior neck; can also occur on face or scalp
Shawl sign: macular rash over posterior shoulders, neck
PM/DM: Constitutional sx: fever, malaise, weight loss, Raynaud’s phenomenon (may have Raynaud for yrs before other sx; then Raynaud remits & other sx show up)
PM/DM: Musculoskeletal: symmetric arthritis, joint contractures
PM/DM: GI: esophageal dysmotility (also stomach, small bowel), dysphagia
PM/DM: Cardiac: conduction abnormalities, dilated cardiomyopathy
PM/DM: Skin: calcinosis
PM/DM: Pulmonary: interstitial lung disease, pneumonitis
Inclusion Body Myositis (IBM): Epi In pts >50yrs: most common inflam myopathy; M 2X F
IBM: Clinical presentations: Insidious onset; slow prog; weakness & atrophy of distal mx (calves, foot & finger flexors); present w/ weak grip, foot drop or tripping; weak quads: present w/ falls, impaired mobility; Poor response to treatment modalities
Idio Inflam Myo: Clin findings sig: pt c/o weakness; strikingly elev CK; poss high LDH, aldolase; Abnml EMG (nonspecific but classic)
definitive diagnostic test for Idio Inflam Myo = Mx bx; inflammatory infiltrate; Autoantibodies
Inflam myopathies: ANA Ab 50% prev; High titers usually indicate presence of another CTD
Inflam myopathies: Anti-Jo Ab 25% prev; PM or DM & ILD, arthritis, Raynaud’s
Inflam myopathies: Anti-PM-Scl low prev; Ab to nucleolar protein complex; assoc with PM/SSc
Inflam myopathies: Anti-Mi-2 low prev; DM with V sign, shawl sign
Idio Inflam Myo: mainstay of tx = Glucocorticoids: oral prednisone start high (60mg/d); taper as CK normalizes
Idio Inflam myopathies: Tx steroids; screen for malignancies
Idio Inflam myopathies Other immunosupp tx: azothioprine, MTX, cyclophosphamide, mycophenolate mofetil
Idio Inflam Myo: Immunomodulators IV immunoglobulin: 2g/kg over 2-5 days
IBM: Tx generally non-responsive to immunosuppresive tx
Scleroderma: Pathogenesis: defect in fibroblast metabm: fibrosis of skin & internal organs (lungs, kidneys, heart, GI tract); vascular dysfunction; (auto)immune cell activation; interaction among the 3
Scleroderma: vascular dysfunction (vasculopathy) Raynaud; digital ischemia, infarction
Scleroderma: immune cell activation increased levels of cytokines & T-cell receptor molecules; disease specific autoantibodies
Scleroderma Epidemiology F 3-4 x M; US: 10-20/ million; age, gender & ethnic sig factors in suscept; more common in AA pts; usu 3d / 4th decade
Scleroderma: Environmental factors: viral infection; silica
Scleroderma: vasculopathy: earliest manifestation = Raynaud’s phenomenon
Scleroderma: Cellular / humoral autoimmunity: Early in dz, monos & T cells accumulate in skin & affected organs
Scleroderma: Circulating autoantibodies present in nearly all pts; highly specific for sclero; exact role in pathogenesis unclear
Scleroderma: Fibrosis: progressive replacement of normal tissue w/ dense conn tissue: leads to exuberant matrix remodeling & scar formation; fibrosis dev in skin, lungs, tendon sheaths, heart, GI tract, some endocrine organs; functional impairment
Sclerodactyly = localized thickening / tightness of skin of the fingers or toes
CREST syndrome: assoc w/lcSSc; calcinosis, Raynaud; esophageal dysmotility sclerosis & telangiectasias
Scleroderma: Skin: lcSSc limited to face, fingers and toes
Scleroderma: Skin: dcSSc fibrosis is widespread
Scleroderma: Lungs: leading cause of death in dcSSc = interstitial disease
Scleroderma: GI tract: dysmotility of esophagus, stomach, small bowel; GERD sx develop early
Scleroderma: MS periph neuropathies; genl arthralgias (hands, wrists); tendon rubs; myositis (dcSSc, not common in lcSSc)
Scleroderma: ANA prev 95%; both lcSSc & dcSSc
Scleroderma: anti-topoisomerase (Scl-70) more assoc w/diffuse dz; assoc w/ tendon rubs, pulmo fibrosis, inc mortality
Scleroderma: anti-centromere more in LcSSc; assoc w/ CREST, pulmonary HTN, Raynaud
2 autoAbs unique to scleroderma: anti-topoisomerase (Scl-70); anti-centromere
Scleroderma: Tx tx manifestn (eg, Raynaud)
Scleroderma: Tx: GI sx: PPI /H2 blockers, Abx, prokinetics; frequent small meals
Scleroderma: Tx: Vasculopathies: Raynaud: smoking cessation, CCBs, topical nitroglycerin, losartan, prazosin; statins: delay progression?
Scleroderma: Tx: Cardiopulmonary: tx right-sided failure, pulmonary HTN; O2; cyclophophosphamide; trepotinil
Polymyositis tx HD steroids, MTX, or azathioprine until sxs resolve
Scleroderma tx: renal ACEI
DM/PM S/S: progressive, symmetric proximal weakness (neck, pharynx, pelvis/thighs, shoulder); inc mx enzymes; histologic changes (inflam infiltrate) in striated mx tissue
PM/Dermatomyositis dx assoc with: assoc w/occult malig
Dermatomyositis cutaneous sx heliotrope; Gottron’s papules; Gottron’s sign; V sign; Shawl sign
Dermatomyositis: heliotrope: erythematous or violaceous macular rash of one or both eyelids; usually accompanied by edema
Dermatomyositis: Gottron’s papules: sl raised pink, dusky red or violaceous papules over dorsal sides of MCP/PIP and/or DIP joints. Can also occur over wrists, elbows or knees
PM/DM: Pulmonary: interstitial lung disease, pneumonitis
Inclusion Body Myositis: Clinical presentations: Insidious onset; slow prog; weakness & atrophy of distal mx (calves, foot & finger flexors); weak grip, foot drop or tripping; weak quads: falls, impaired mobility; Poor response to tx
Scleroderma: Pathogenesis: defect in fibroblast metabm: fibrosis of skin & internal organs (lungs, kidneys, heart, GI tract); vascular dysfunction; (auto)immune cell activation; interaction among the 3
Scleroderma: vascular dysfunction (vasculopathy) Raynaud; digital ischemia, infarction
Scleroderma: immune cell activation increased levels of cytokines & T-cell receptor molecules; disease specific autoantibodies
Scleroderma: Environmental factors: viral infection; silica
Scleroderma: Cellular / humoral autoimmunity: Early in dz, monos & T cells accumulate in skin & affected organs
Scleroderma: Skin: hallmark is: fibrosis of skin
Scleroderma: Skin: lcSSc vs dcSSc lcSSc (limited cutaneous systemic Sc): limited to face, fingers and toes; dcSSc: fibrosis is widespread
Created by: Abarnard
 

 



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