Second Department of Internal Medicine, Saitama Medical School.
We reported two sisters of primary biliary cirrhosis (PBC)-CREST overlap syndrome complicated with Sjögren's syndrome (SS). Both patients had Raynaud's phenomenon, sclerodactylia, telangiectasia, chronic sialoadenitis, chronic nonsuppurative destructive cholangitis by liver biopsy and were positive for anti-centromere antibodies. This is the first report of two sisters of PBC-CREST overlap syndrome complicated with SS.