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21 Cards in this Set
- Front
- Back
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types of scleroderma?
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systemic: diffuse, limited (CREST)
localized: linear, morphea |
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epidemiology of scleroderma?
incidence sex age |
5-10/million/yr
MF: 1:4 age 30-50 |
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presentation of scleroderma
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puffy phase -> tight phase hand
digital telangiectasia and acroosteolysis interstitial lung disease renal crisis ANA excess dermal colagen digital calcinosis raynaud's digital pitting scars nailfold capillary dilation esophageal dysmotility |
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prevention precaution of raynaud?
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stay warm..
quit smoking |
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Tx of interstitial lung disease in scleroderma?
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immunosuppressants
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Tx of renal crisis in scleroderma?
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ACE inhibitor
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differentiation of CREST from diffuse scleroderma?
skin telangiectasia calcinosis tendon rub pulmonary HTN renal crisis Anti centromere |
CREST vs diffuse
skin: distal - proximal/distal telangiectasia: +++ - + calcinosis:: +++ - + tendon rub: 0 - +++ pulmonary HTN: ++ - 0 renal crisis: 0 - ++ Anti centromere: +++ - 0 |
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survival rate limited vs diffuse scleroderma?
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limited vs diffuse
1yr: 98% - 80% 6 yr: 80% - 30% 12 yr: 50% - 15% |
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problem oriented management of scleroderma
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raynaud: dress wam. no tobacco, vaso dilator
arthritis/serositis: NSAID, low dose steroid heartburn/reflux: H2 blocker, PPI, promotility renal crisis: ACE inhibitor Pulm HTN: endothelin receptor antagonist (bosentan), prostacyclin, sildenafil malabsorption: flagyl, cipro, TCN loss of mobility: PT / OT |
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pathogenesis of scleroderma?
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1. microvascular occlusion (via endothelial cell activation)
2. excessive connecive tissue (fibrosis) - via activated dermal and visceral fibroblasts, increased TIMP 3. immune cell activation - enhance fibroblast and endothelial processes |
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definition of
myositis? polymyositis? dermatomyositis? |
myositis? inflammation of muscles
polymyositis? acquired diffuse inflammatory disorder of skeletal muscle dermatomyositis? polymyositis accompanied by characteristic rash |
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epidemiology of PM/DM
incidence sex ratio age |
5/million / year
MF (adult): 1:2 MF (children): 2:1 age - bimodal 5-15, 45-65 |
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characteristics of muscle weakness in PM/DM?
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proximal > distal, symmetric
insidious onset dysphagia, dysphonia |
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clinical presentations of PM/DM?
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heliotrope rash in child DM
calcinosis in child DM shawl-pattern rash in adult DM gottron's papule in adult DM (MCP, PIP usually, occasionally knee) prominent failfold capillaries |
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lab study in PM/DM?
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EMG changes. increased muscle enzymes (CPK, aldolase), muscle biopsy, positive ANA
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which serum enzyme most sensitive in PM/DM?
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CPK.
Tx: high dose steroids |
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what's seen in muscle biopsy in PM/DM?
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positive in 80-90%
degenerating/regenerating myofibers interstitial lymphocytic infiltration fibrosis, atrophy DDx: inclusion myositis |
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what are myositis specific autoantibodies and their clinical significance? (3 of them)
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1. anti-synthetase: Jo-1 (histidy-tRNA synthetase) - manifests as interstitial lung disease, fever, arthritis, mechanic's hand
2. anti- signal recognition particle (SRP): acute onset severe myositis, steroid resistant 3. anti-Mi-2 (helicase): classical dermatomyositis |
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Tx for PM/DM?
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begin w/ prednisone 60-100mg/day
taper 25% of dose every 3-4 wks if CPK normal continue low-dose (10-15mg) prednisone for 1 yr beware steroid myopathy azathioprine, methotrexate, experimental therapies for resistant disease |
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inflammatory mechanisms in polymyositis?
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1. cell mediated antigen specific cytotoxicity ( T cells)
2. humorally mediated muscle fiber damage (perivascular B cell infiltrate, deposition of membrane attack complex) |
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abnormalities of myocyte fxn?
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1. destruction of muscle fibers, fibrosis
2. disordered ATP dependent metabolism disrupts muscle contraction, membrane integrity 3. TNF inhibits contraction, promotes catabolism 4. capillary dropout, hypoperfusion result: muscle weakness |
