[Primary biliary cirrhosis and scleroderma: long-term benign course of a complex autoimmune disease]

A 57 year old female patient suffered from Raynaud's syndrome and relapsing necrotizing lesions of the finger tips. Diagnosis of sclerodermia was established by angiography and biopsy. A spotty brownish coloration of the skin and cirrhosis of the liver were observed in addition. Clinical chemistry showed increased alkaline phosphatase and increased titers of antimitochondrial antibodies. Histology of liver tissue was compatible with a diagnosis of primary biliary cirrhosis. After a course of several years a Sicca syndrome appeared. On investigation of family members an increased incidence of positive antinuclear and antimitochondrial antibodies was found. The patient has now been followed for 5 years. Prognosis may be considered to be good.