A Case of Primary Biliary Cirrhosis in Association with an Overlap Syndrome of Systemic Sclerosis and Systemic Lupus Erythematosus / 대한류마티스학회지

ABSTRACT

Primary biliary cirrhosis (PBC) is a slowly progressive autoimmune disease of the liver that is related to anti-mitochondria antibody and the disease is characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. Several autoimmune diseases, such as hypothyroidism, Sjogren syndrome and systemic sclerosis (SSc), occur with increased frequency in patients with PBC. However, there are a few reports of a possible connection between systemic lupus erythematosus (SLE) or autoimmune hemolytic anemia (AIHA) and PBC. A 52-year-old female was admitted with fatigue and dyspnea that she had suffered with for the past month. She had suffered from jaundice for 2 weeks before admission. Many of the clinical manifestations and laboratory findings suggested the diagnosis of PBC with SSc of the limited type and AIHA. She was treated with methylprednisolone pulse therapy and ursodeoxycholic acid. We consequently diagnosed her as having SLE, as she satisfied the 4 relative diagnostic criteria-arthritis, AIHA, positive antinuclear antibody and positive antiphospholipid antibodies.