Scleroderma BB
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- 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