Scleroderma

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• aka systemic sclerosis: stiff hard tissue

• most autoimmune: tissue destrcution

• scleroderma: fibroblast

• limited aka CREST

Diffuse

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• people will have raynaud by itself at first

• heart: RBBB, LBBB, AV nodal

• interstitial lung: fibrous tissue in lung

• early in disease

Limited

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• 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

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• crest = centromere

• rarely treated with anti inflammatory drugs

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• sjogren: half of pts have sjogren