Primary biliary cirrhosis and the CREST syndrome: a report of 22 cases

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.