A case of primary biliary cirrhosis complicated with progressive systemic sclerosis, sjögren's syndrome and Hashimoto's thyroiditis

TANAKA Eiji, IMAI Toshisuke, NOSAWA Keiichi, SUSUKI Yoichi, AKAHANE Yoshihiro, KIYOSAWA Kendo, FURUTA Seiichi

doi:10.2957/kanzo.22.1473

Fifty-four year-old woman had been complained of palmar iching, Raynaud's phenomenon and noted abnormal liver function tests and also hypothyroidism over several years. She had been administered prednisolone and desiccated thyroid for these illnesses. At 54 years of age, she developed an overt jaundice and was referred to our clinic. Her physical findings and laboratory datas were compatible for the diagnosis of progressive systemic sclerosis, Sjogren's syndrome and Hashimoto's chronic thyroiditis. These diagnoses were confirmed by histological findings of biopsied specimens of thyroid gland, salivary gland and skin. As regards the jaundice, her liver function tests showed the jaundice of cholestatic type, but ERCP findings ruled out the extrahepatic obstruction. Anti-mito-chondrial antibody in her serum was negative on repeated tests. Serial sections of wedge-biopsied liver obtained under surgical laparotomy revealed chronic non-suppurative destructive cholangitis, compatible for primary biliary cirrhosis. The underlying mechanisms of these complications of wide varieties in primary biliary cirrhosis were discussed.

A case of primary biliary cirrhosis complicated with progressive systemic sclerosis, sjögren's syndrome and Hashimoto's thyroiditis

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