Advances in classification, prognostication and treatment of immunocholangitis

Immullocholangitis is a collective for chronic inflammatory disorders affecting the biliary tree, presumably with an autoimmune-mediated pathogenesis. Destruction and distortion of bile ducts, leading to impaired bile flmv, are key features of immunocholangitis. In general, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are considered to be the main diseases of immunochoiangitis. PBC, a chronic cholestatic liver disease, is one of the most common vanishing bile duct disorders. Gradual loss of interlobular and septal bile ducts, histologically described as chronic non-suppurative destructive cholangitis, leads to chronic cholestasis, fibrosis and biliary cirrhosis which may ultimately cause liver failure, necessitating transplantation. Since 1988, PBC has been the third leading indication for liver transplantation in Europe (European Liver Transplant Association (ELTA) registration). Typical symptoms of patients with PBe are pmritus, fatigue, arthralgia and dryness of the eyes and mouth. A large proportion of patients, especially those with early stage PBC are however often asymptomatic. Physical examination may reveal jaundice, scratch marks, xanthelasma and xanthoma, in particular in the event of marked cholestasis. PBC is a relatively rare disorder in comparison with chronic viral hepatitis and alcoholic liver disease. It affects primarily middle-aged (40-60 years) women (male:female ratio 1:9). In the 1970's, reported prevalences ranged from only 18 to 54 cases per one million inhabitants. Since then, substantially higher prevalence rates have been reported. Recent studies in Wales and the Newcastle area revealed a prevalence of 200-250 PBC patients per one million inhabitants in 1994, suggesting a more than tenfold increase in less than 20 years! Whether these data reflect a true increase in the number of cases is uncertain. The development of more sophisticated epidemiological case fmding methods, increased awareness of PBC as a possible cause of chronic liver disease and routine assessment of serum liver tests for medical examinations (i.e. for insurance) may all have contributed to the identification of more, in particular asymptomatic, cases. Moreover, the diagnosis of asymptomatic subjects might result in higher prevalence rates because of longer survival. PSC is a cryptogenic chronic cholestatic liver disease characterised by progressive strichlring and obliteration of intrahepatic andlor extrahepatic bile ducts, leading to fibrosis and biliary cirrhosis which may cause hepatic decompensation. Since 1988, PSC has been the sixth leading indication for transplantation (ELTA registration). The signs and symptoms of PSC resemble those of PEe. PSC is regarded as an even more rare disease than PBC, but reliable epidemiological data on prevalence and incidence are lacking. Data from Sweden suggest a prevalence of 60-80 patients per one million inhabitants. There is a strong association with inflammatory bowel disease (IBD). It has been found that approximately four percent of patients with IBD have concomitant PSC. Conversely, 50-70% of patients with PSC also have IBD. PSC has a male predominance (male:female ratio 2: 1). The median age at diagnosis is 35 years.