A case of primary biliary cirrhosis associated with Sjøgren's syndrome and sclerodactylia

Abstract

A 54 year-old female case of primary biliary cirrhosis (PBC) with typical Sjgren's syndrome and sclerodactlyia is described.
Initial symptom of Raynaud's phenomenon since 10 years before was followed by fluctuating jaundice during recent five years. On admission marked jaundice of obstructive ytpe with elevated serum transaminase, notable increased serum alkaline-phosphatase (603 IU), increased seurm IgM (343mg) and strong positive antimitochondrial antibody was strongly suggestive of PBC. The diagnosis was determined by the histology of wedge-biopsied specimen of the liver. Additionally coexistence of typical Sjgren's syndrome by parotid sialography, lip salivary gland biopsy and Schirmer's test, and of sclerodactylia by finger skin biopsy was confirmed.
Immunological cross reactivity between the bile duct system and salivary duct system might be considered for the pathogenesis in this case.