A patient with CREST syndrome (calcinosis cutis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) who had severe jaundice (total bilirubin 29.1 mg/dl) and rapidly progressive liver damage is reported. The liver damage findings matched the criteria of autoimmune chronic active hepatitis (CAH). There have been no prior reports of a case of CREST syndrome with autoimmune CAH in Japan. Anticentromere antibody (ACA) was detected in the serum; ACA seemed to be related to the pathogenesis of these two diseases.

CREST syndrome can be associated with rapidly progressive liver damage, suggesting a potential link between autoimmune chronic active hepatitis and CREST syndrome.

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A case of CREST syndrome with rapidly progressive liver damage.

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