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Polymyositis and Acute Onset Systemic Sclerosis in a Patient with Primary Biliary Cirrhosis: A Clinical Syndrome Similar to the Mixed Connective Tissue Disease

Primary biliary cirrhosis (PBC) is a multisystem disease, characterized by cholestatic jaundice, and an association with other 'collagen' diseases as described by Sherlock & Scheuer (1973) and Schaffner (1979). The diagnosis is based on a positive mitochondrial antibody (MA) test, increased levels of serum IgM and a diagnostic or compatible liver biopsy (Sherlock & Scheuer 1973). The mixed connective tissue disease syndrome (MCTD) characteristically affects mainly females and has clinical features of systemic lupus erythematosus (SLE), systemic sclerosis and polymyositis (Ellery 1972, Sharp et al. 1972, Bresnihan et al. 1977). The serologic findings are a high titre to antinuclear antibody (ANA) in a speckled pattern and ahigh haemagglutination titre of antibody to extractable nuclear antigen (ENA). Invariably this antibody is directed against the RNase-sensitive ribonucleoprotein antigen (RNP) and not against the RNaseresistant Sm antigen (Sharp et al. 1976). This report describes a young woman who presented with PBC and subsequently developed polymyositis and two years later rapidly progressive systemic sclerosis. One other case has previously been reported (Uhl et al. 1974). Both the myositis and the systemic sclerosis responded rapidly to treatment with prednisolone. The clinical features of this syndrome resembled those of MCTD but the autoantibody profile was different from that previously described for MCTD (Ellery 1972, Sharp et al. 1972, 1976, Bresnihan et al. 1977). It is suggested that PBC may be associated with a syndrome similar to MCTD.