Scleroderma

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Basics

Description

  • 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
    • Limited
      • 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)

Geriatric Considerations
Uncommon >75 years of age

Pediatric Considerations
Rare in this age group

Pregnancy Considerations
  • 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.

Epidemiology

Incidence
  • 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)

Prevalence
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).

  • Unknown
  • 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.

Genetics
Familial clustering is rare but has been seen.

Risk Factors

Unknown

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