Clinical and Biological Characteristics of Algerian Patients with Primary Biliary Cholangitis (PBC)

Abstract

Background. Primary Biliary Cholangitis (PBC) is a rare chronic autoimmune hepatopathy characterized by progressive destruction of the small-caliber intrahepatic bile ducts. It is an essentially cell-mediated disease, but autoantibodies associated with the disease are particularly useful for diagnosis. Objective. We aimed to study the clinical and the biological characteristics of Algerian PBC patients. Materials and methods. This is a prospective study, performed at Blida University Hospital Center – Algeria, ranging from January 2017 to December 2018, involving a cohort of 17 patients with PBC aged between 20 and 75 years. Serum M2 anti-mitochondria autoantibodies (AMA-M2) were tested by indirect immunofluorescence (IIF) on triple substrate, by IIF on Hep-2 and by enzyme-linked immunosorbent assay (ELISA). Results. The mean was 50.2 years. A female predominance was found with a sex ratio (F:M) of 7,5: 1. The most frequent clinical sign was asthenia, followed by arthralgia and icterus. Furthermore, 70.6% of the patients studied had at least one associated autoimmune disease. Biologically, 41.6% had isolated cholestasis and 58.4% had a combination of cholestasis and cytolysis. All patients had a significant titer of AMA-M2 (≥ 1/40th) on triple substrate. Conclusion. In this study, the clinical symptomatology is marked mainly by asthenia and arthralgia as well as icterus, which are non-specific signs of PBC. Autoimmune thyroiditis was the most frequent associated pathology to CBP. Biologically, cholestasis is one of the essential criteria for the diagnosis of PBC. The detection of AMA-M2 remains the main element that confirms the diagnosis of PBC; nevertheless, other autoantibodies were detected such as ANA, SMA and anti-LKM.