Primary Biliary Cholangitis as the Initial Manifestation of Systemic Lupus Erythematosus: A Case Report

Abstract

Systemic lupus erythematosus (SLE) is a complex autoimmune disease affecting multiple organs, commonly seen in women of childbearing age. Its incidence varies globally, ranging from 1 to 10 cases per 100,000 person-years. Liver involvement in SLE is relatively rare, occurring in 3-9% of cases. Primary biliary cholangitis (PBC), a chronic autoimmune liver disease, leads to bile duct destruction, cholestasis, and, if untreated, liver cirrhosis. The global prevalence of PBC is 14.6 per 100,000 persons, with lower rates observed in the Asia-Pacific region.   PBC can coexist with SLE, though rare, with a reported prevalence of 0.27% among SLE inpatients. PBC often precedes SLE diagnosis in patients with both conditions and is associated with more severe disease manifestations and complications. 

A 20-year-old woman presented with jaundice, abdominal pain, and fever, alongside a history of acute cholestatic liver failure and kidney injury. Investigations revealed liver cirrhosis, confirmed PBC through antibody testing, and severe SLE. Treatment included steroids, hydroxychloroquine, ursodeoxycholic acid (UDCA), and supportive therapies, resulting in significant clinical improvement.

Clinicians should consider the possibility of overlapping autoimmune diseases such as PBC and SLE, especially in young women presenting with cholestasis and systemic symptoms.