A case of mixed connective tissue disease/Sjoegren's syndrome associated with primary biliary cirrhosis.

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The patient is a 54-year-old female. Massive hair loss and Raynaud's phenomenon were noticed at the age of 35 and 37. She was found to have abnormal liver function and high value of ZTT at the age of 40 and 48, respectively. She was sent to the Keigu clinic because of a positive ANA determination. Her complaints were massive hair loss, sausage like finger and dry mouth, and her blood chemistry was in the normal range except for 8.6g/dl of T.P. and slight elevation of ALP. On the immunological examination, she was found to have a variety of auto-antibody such as AMA (1280), anti-M2 (over 200 U/l by ELISA), ANA (5120 speckled type), anti-SS-A (16), anti-nRNP (8), anti-Tu and anti-MM antibodies. Liver biopsied specimen revealed epithelial cell destruction, detachment and degeneration of the moderate cholangial duct and small nuclear cell infiltration arround these duct, suggesting the stage of florid bileduct lesion of chronic non suppurative cholangitis (CNSDC). We reported here a case of mixed connective tissue disease/Sjögren's syndrome associated with primary biliary cirrhosis. Furthermore, we could analyze most of autoantibodies found in this case when determined by the immunoprecipitation.