Paris Criteria Are Effective in Diagnosis of Primary Biliary Cirrhosis and Autoimmune Hepatitis Overlap Syndrome

Abstract

Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) differ in clinical, laboratory, and histologic features as well as in response to therapy. A small subgroup of patients have an overlap syndrome with features of both diseases, although there is no consensus on its definition or diagnostic criteria. We evaluated the significance of the criteria used to diagnose PBC-AIH overlap syndrome. This retrospective, single-center study included all patients diagnosed with PBC, AIH, or PBC-AIH overlap syndrome, based on the Paris criteria, since January 1990 (n = 134); patients were followed up for 9.7 +/- 3.7 years. The 3 groups were compared for their clinical, laboratory, and histologic features. Patients with overlap syndrome or PBC were graded by the revised and simplified AIH scoring systems to assess the ability of this system to identify AIH cases properly. The sensitivity and specificity of the Paris criteria for diagnosing the overlap syndrome were 92% and 97%, respectively. The sensitivity and specificity of the AIH scoring systems were considerably lower. Among patients with the overlap syndrome, the 10-year, transplantation-free survival rate was 92%. The Paris diagnostic criteria detect overlap syndrome (PBC and AIH) with high levels of sensitivity and specificity. The clinical value of the revised and simplified AIH scoring system is not as reliable. Patients with PBC-AIH overlap syndrome have a 92% rate of 10-year, transplantation-free survival.