Scleroderma (Progressive Systemic Sclerosis, PSS)

Characterized by excessive fibrosis in a variety of tissues from collagen deposition by activated fibroblasts. About 75% of cases are in women, mostly middle aged. Patterns of disease include:

Limited scleroderm, or CREST syndrome: the benign form of PSS, serologically suggested by the presence of anti-centromere antibody

  • C = Calcinosis in skin and elsewhere
  • R = Raynaud's phenomenon, sensitivity to cold
  • E = Esophageal dysmotility from submucosal fibrosis
  • S = Sclerodactyly from dermal fibrosis
  • T = Telangiectasias

Diffuse scleroderma: the worst form of PSS; Scl-70 (anti-DNA topoisomerase I) antibody shows specificity for this form. It may include all of the findings with CREST, but additionally has renal findings: arterial intimal thickening and proliferation (hyperplastic arteriolosclerosis) leading to hypertensive emergency (malignant hypertension) when systolic pressure is ≥180 and/or diastolic pressure ≥120 mmHg along with signs of acute or ongoing end-organ damage with arterial fibrinoid necrosis, thrombosis, and renal infarction. Half of diffuse PSS patients die from renal disease. The lungs in this form of scleroderma may have diffuse alveolar fibrosis leading to honeycomb fibrosis

Morphea: this is skin fibrosis only