Systemic sclerosis

From IDWiki

Syndromes

  • Scleroderma (only skin involvement)
  • Limited cutaneous systemic sclerosis (lcSSc)
    • CREST syndrome
  • Diffuse cutaneous systemic sclerosis (dcSSc)
  • SSc sine scleroderma (only internal organ involvement)
  • Environmentally-induced scleroderma (vinyl chloride, epoxies, pesticides)
  • Overlap syndromes

Limited cutaneous systemic sclerosis (lcSSC)

  • Sclerosis often restricted to hands, face, and neck
  • May have the CREST syndrome
    • Calcinosis cutis
    • Raynaud phenomenon
    • Esophageal dysmotility
    • Sclerodactyly
    • Telangiectasia

Diffuse cutaneous systemic sclerosis (dcSSc)

  • Extensive skin sclerosis, including proximal to wrists, including proximal limbs and trunk
  • Greater risk for internal organ involvement, including kidneys, lungs, and heart

Management

Raynaud phenomenon

  • Non-pharmacologic: smoking cessation, cold avoidance
  • First-line: nifedipine or other DHP calcium channel blocker
  • Second-line: PDE-5 inhibitors, such as sildenafil, tadalafil, or vardenafil
  • Non-guideline second-line: topical nitroglycerine
  • Can consider fluoxetine
  • Last-line: IV iloprost or other prostanoid

Further Reading