Primary biliary cirrhosis in Israel

Primary biliary cirrhosis (PBC) is a relatively rare autoimmune disorder leading to the destruction of the interlobular biliary epithelium, which has not been reported in the Middle East. We studied 30 patients with PBC who had been referred to the Liver Unit at the Hadassah Medical Center in Jerusalem. The diagnosis was established by conventional criteria in 28 female and 2 male patients. Twenty-two patients were of Ashkenazic origin and 8 of Sephardic background. Mean serum alkaline phosphatase activity at the time of diagnosis was 911 IU/l gamma-glutamyl transpeptidase 677 u/l, cholesterol 73 mmol/l, albumin 3.2 g/l, bilirubin 72 mmol/l, and prothrombin time was 65%. All patients had positive antimitochondrial and M2 antibodies, and the mean IgM level was 684 mg/dl. The diagnosis was confirmed by liver biopsy in 27 of 30 patients. To the best of our knowledge this represents the first report of primary biliary cirrhosis in the Jewish population in Israel. This retrospective survey raises the question whether the disease is indeed rare in Israel or, alternatively is underdiagnosed.