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🌱 來自: systemic sclerosis and scleroderma disorders

Definition and epidemiology of systemic sclerosis and scleroderma disorders

• Scleroderma refers to the presence of tight, thickened skin
• Localized scleroderma: morphea (plaques of fibrotic skin), linear (fibrotic bands), “en coup de sabre” (linear scleroderma on one side of scalp and forehead ≈ saber scar)
• Systemic sclerosis (SSc) = scleroderma + internal organ involvement. High-mortality.
  • SSc w/ limited cutaneous disease (lcSSc): formerly CREST syndrome (see below)
  • SSc w/ diffuse cutaneous disease (dcSSc): often rapidly progressive skin thickening
  • SSc sine scleroderma (visceral disease without skin involvement, rare)
• Peak onset age 30-50; ♀ >♂ (8:1). Earlier/more severe disease in African Americans
• <6/100,000 annual SSc incidence wordwide; lcSSc incidence ~2× that of dcSSc
• Pathogenesis: unclear. Endothelial injury → ROS/oxidative stress → perivascular inflammation → fibrosis. Cytokines, growth factors, genetics, environ. factors + antibodies (against PDGFR, endo. cells, fibroblasts) may contribute (NEJM 2009;360:1989).