VIÊM ĐƯỜNG MẬT TIÊN PHÁT PHỐI HỢP HỘI CHỨNG SJOGREN: CA LÂM SÀNG

Abstract

Primary biliary cholangitis is a chronic autoimmune liver disease and is relatively rare. It is caused by the destruction of the small intrahepatic bile ducts, leading to cholestasis, inflammation, and eventually cirrhosis. The disease is characterized by the presence of anti-mitochondrial antibodies (AMA), is associated with autoimmune mechanisms, and is often accompanied by other autoimmune disorders. Sjögren’s syndrome is commonly observed in patients with primary biliary cholangitis, with a prevalence ranging from 7-34 %, presenting with symptoms such as dry eyes, salivary gland swelling, joint involvement, myalgia, muscle weakness, pruritus, cutaneous vasculitis, Raynaud’s phenomenon, respiratory involvement, and neurological manifestations. We describe the case of a 46-year-old female patient who had been diagnosed with and treated for primary biliary cholangitis for many years, with positive anti-mitochondrial antibodies, in association with Sjögren’s syndrome. Her clinical manifestations included dry mouth and cold-induced pain in the second fingers of both hands, along with positive antinuclear antibodies: SSA, Ro-52, gp210, and Mi-2β antibodies. The patient’s disease was controlled with a combination of ursodeoxycholic acid, fibrates, and comprehensive care.