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Scleroderma
Rheumatology
| Question | Answer |
|---|---|
| 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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