Primary biliary cirrhosis and the crest syndrome: New terminology?

Abstract

The syndrome of primary biliary cirrhosis and limited scleroderma (the CREST syndrome) is described in 22 patients. The syndrome is expanded to include a serologic marker—the anticentromere antibody—and keratoconjunctivitis sicca. The acronym “PACK” (primary biliary cirrhosis, anticentromere antibody, CREST syndrome, and keratoconjunctivitis sicca) with “syndrome” is suggested to facilitate recall of the various components of this entity. Copyright © 1988 American Association for the Study of Liver Diseases