Progressive Systemic Sclerosis

 

Progressive systemic sclerosis
-widespread fibrosis and degenerative changes involving many organs
-fixed facial appearance, sclerodactyly (claw-like hand), Raynaud phenomenon, visceral organs involvement (esophagus-dysphagia, lung-internsitial pulmonary fibrosis, hypertension).
-anti-Scl-70, ANAs with anticentromere activity (in CREST syndrome)
-CREST syndrome: caclinosis, raynaud phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia

 

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