Primary biliary cirrhosis: underdiagnosed pathology. Case report

Abstract

Introduction: Primary biliary cirrhosis is a chronic, cholestatic, autoimmune, and progressive liver disease that predominantly affects women. It is characterized by inflammation and progressive destruction of the interlobular bile ducts and insidiously presents with increased liver function tests and excretion. Case description: A 70-year-old female patient, diabetic, hypertensive, and hypothyroid, was treated at the National Diabetic Institute. Elevated transaminases and bilirubin levels were observed. Additionally, she had dyslipidemia managed with statins, wich were discontinued due to the associated liver damage. The patient was managed with statins, which were omitted due to the associated liver damage. The only symptoms were mild pain in the right upper quadrant and asthenia, with not jaundice, pruritus, or fever. Ultrasounds revealed hepatic steatosis. Autoimmune disease was investigated, resulting in elevated C-reactive protein and erythrocyte sedimentation rate titers. Anti-nucleus cytoplasmic antibodies by indirect immunofluorescence positive and antimitochondrial antibodies positive. The Pathology report showed biliary pattern lesion, chronic portal inflammation, florid ductal lesion, nodular regenerative hyperplasia, grade 1 macrovesicular steatosis. With the analysis of the data, the definitive diagnosis of primary biliary cirrhosis is provided and referred to Gastroenterology. Conclusions: Primary biliary cirrhosis is a pathological entity that requires high diagnostic suspicion, in addition to the interpretation of blood biochemistry studies, immunological studies, guide us to consider possible differential diagnoses. The definitive diagnosis is made by liver biopsy and positive antimitochondrial antibodies.