Final Prep - Musculoskeletal - Scleroderma Flashcards


uncommon connective tissue disease hallmark:
overproduction & accumulation of collagen & other
extracellular matrix proteins -> fibrosis, tissue
dysfunction more common in African Americans, Hispanics,
Native Americans


Reynaud's - primary Reynaud's & auto-immune
Reynaud's, common excessive vasoconstriction in extremities
localized - morphea, linear scleroderma (sclerotic
lesions appearing as linear bands)
systemic: limited cutaneous -
most common, skin thickening in areas distal to elbows & knees,
CREST syndrome (Calcinosis-Reynaud's-Esophageal
dysfunction-Sclerodactyly-Telangletasias), diffuse cutaneous
abnormalities of vasculature of mult. organ systems

Diffuse Scleroderma Sx

pulmonary: interstitial lung disease, pulmonary
vascular disease
cardiac: pericarditis, pericardial effusion,
myocardial fibrosis, CHF, myocarditis, arrhythmias
GI: GERD, altered motility, strictures, gastric
antral vascular ecstasia
Renal: kidney failure


CBC ESR / CRP auto-antibody screen
24hr urine Cr clearance microscopy mid-stream
urine GFR CSR PFT CT scan
electrocardiography echo skin biopsy


no cure organ-specific meds
immunosuppressants phototherapy (UVA irradiation)


Ineffective Peripheral Tissue Perfusion Impaired
Physical Mobility Impaired Skin Integrity