Scleroderma BB

  • aka systemic sclerosis: stiff hard tissue

  • most autoimmune: tissue destrcution
  • scleroderma: fibroblast

  • limited aka CREST

Diffuse

  • people will have raynaud by itself at first
  • heart: RBBB, LBBB, AV nodal

  • interstitial lung: fibrous tissue in lung

  • early in disease

Limited

  • CREST can occur in diffused but main symptoms of limited

  • difficulty swallowing: collagen deposition in esophagus
  • LES: collagen deposition disrupts LES activity. Tone measured with esophageal monometry
  • achalasia: hypertonicity

  • women: especially on cheeks and forehead

Diagnosis

  • crest = centromere

  • rarely treated with anti inflammatory drugs

  • sjogren: half of pts have sjogren