Systemic Sclerosis

Definition: Autoimmune conditions associated with widespread fibrosis of organs, secondary to overproduction of collagen and other extracellular matrix proteins.

Epidemiology: 50 - 60 yo; f:m - 3:1

Clinical features:

• • Raynaud's phenomenon (vasospasm of arteries in hands in response to cold or emotional stress, resulting in discoloration of hands.

  • Thickened, tight skin

  • Dysphagia due to esophageal fibrosis

  • Renal artery fibrosis

  • Pulmonary HTN

  • Telangiectasia

  • Limited scleroderma (a.k.a CREST syndrome): Calcinosis cutis, Raynaud's phenomenon, Esophageal dysfx, Scelrodactyly, and Telangiectasia.

  • Diffsuse scleroderma: Widespread skin involvement, including pulmonary fibrosis resulting in pulmonary HTN, HTN, and renal involvement with oliguric crisis.

Lab: Abs: CREST: anticentromere-ab; diffuse Scleroderma: Anti-Scl70 (against DNA topoisomerase I), ANA, anemia, ESR elevation, reduced VC on PFT (restrictive lung disease).

Tx:

• • Pencillamine - may inhibit collagen cross-linking

  • Captopril: controls renal HTN

  • CCB: diminishes Raynaud's phenomenon

  • Steroids - rarely effective. May cause renal crisis.