Scleroderma

Etiology

  • A family of autoimmune, multi-system, connective tissue d/o
  • Can be limited to the skin alone (localized scleroderma); or have more diffuse symptoms and findings→ systemic sclerosis
  • Systemic sclerosis (SS) broken into limited cutaneous (lcSSc) or diffuse cutaneous forms (dcSSc)
  • SS can have overlap with lupus, rheumatoid arthritis, polymyositis, or Sjögren's

Risk Factors

  • Women > men
  • < 50
  • Known disease

Symptoms

  • Skin tightening
  • Sub-cu calcium
  • Telangiectasias
  • Raynauds
  • GERD due to atrophy of esophageal smooth muscle, leading to impaired peristalsis and decreased LES tone, leading to Barretts (increased risk distal esophageal cancer), scarring, dysphagia
  • If systemic→ can affect lung (DPLD), pericardium, joints, etc
  • Additional symptoms based on organ systems involved: SOB, chest pain, joint pain/swelling

Physical Exam Findings

  • Skin tightening
  • Raynauds → fingers blanch/hurt when exposed to cold temp
  • Dry crackles if interstitial lung disease

Sub-Diseases

Links