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- Scleroderma (systemic sclerosis [SSc]) is a chronic disease of unknown cause characterized by diffuse fibrosis of skin and visceral organs and vascular abnormalities.
- Most manifestations have vascular features (e.g., Raynaud phenomenon), but frank vasculitis is rarely seen.
- Can range from a mild disease, affecting the skin, to a systemic disease that can cause death in a few months
- The disease is categorized into two major clinical variants (1).
- Diffuse: distal and proximal extremity and truncal skin thickening
- Restricted to the fingers, hands, and face
- CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia)
- System(s) affected: include, but not limited to skin, renal, cardiovascular, pulmonary, musculoskeletal, gastrointestinal (GI)
Uncommon >75 years of age
Rare in this age group
- Safe and healthy pregnancies are common and possible despite higher frequency of premature births.
- High-risk management must be standard care to avoid complications, specifically renal crisis.
- Diffuse scleroderma causes greater risk for developing serious cardiopulmonary and renal problems. Pregnancy should be delayed until disease stabilizes.
- In the United States: 1 to 2/100,000 per year
- Predominant age
- Young adult (16 to 40 years); middle-aged (40 to 75 years); peak onset 30 to 50 years
- Symptoms usually appear in the 3rd to 5th decades.
- Predominant sex: female > male (4:1)
In the United States: 1 to 25/100,000
Etiology and Pathophysiology
Pathophysiology involves both a vascular component and a fibrotic component. Both occur simultaneously. The inciting event is unknown, but there is an increase in certain cytokines after endothelial cell activation that are profibrotic (TGF-β and PDGF).
- Possible alterations in immune response
- Possibly some association with exposure to quartz mining, quarrying, vinyl chloride, hydrocarbons, toxin exposure
- Treatment with bleomycin has caused a scleroderma-like syndrome, as has exposure to rapeseed oil.
Familial clustering is rare but has been seen.