[A chronic destructive non-suppurative cholangitis-like disease picture with antinuclear antibodies (immunocholangitis)]

Abstract

Three females had suffered, two for many years, from bouts of a liver disease of unknown origin, which clinically, histologically and biochemically fulfills the criteria of chronic destructive non-suppurative cholangitis. None of the patients had antimitochondrial antibodies, but all had antinuclear antibodies at high titres. Two of them are mother and daughter; the latter has two daughters (aged 10 and 12 years), both of whom have antinuclear antibodies at low titres but no abnormal liver function tests. The female family members of the third patient also had antinuclear antibodies in their serum with normal liver enzyme tests. None of the patients was taking any drugs. Contrary to true chronic destructive nonsuppurative cholangitis (primary biliary cirrhosis), in the three patients immunosuppressive treatment with azathioprine (Imurek) and prednisone (Decortin) was successful. It is suggested that the described disease be called immunocholangitis.