Primary Biliary Cirrhosis (PBC)-CREST (Calcinosis, Raynaud's Phenomenon, Esophageal Dysfunction, Sclerodactyly and Telangiectasia) Overlap Syndrome Complicated by Sjögren's Syndrome and Arthritis

A 36-year-old woman with primary biliary cirrhosis (PBC)-CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly and telangiectasia) overlap syndrome complicated by Sjögren's syndrome and arthritis is reported. She had suffered from Raynaud's phenomenon, sclerodactyly, morning stiffness, arthralgia and sicca symptoms since 34 years of age. She exhibited an increased level of alkaline phosphatase and hyperglobulinemia at 2.8 g/dl without any symptoms, and histological findings from the biopsy specimen of the liver were consistent with those of PBC. Her human leukocyte antigen (HLA) typing was DR8 homozygous. (Internal Medicine 34: 451-154, 1995)