PO:16:240 | Systemic Lupus Erythematosus Presenting as End-Stage Liver Disease: A Case Report and Literature Review

Annamaria Tiso¹, Stefania Caccavelli¹, Giuseppina Caroppi¹, Candida Tarullo¹, Gaetano Serviddio¹ | ¹Policlinico Riuniti Foggia, Italy

Aim of work. Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease with diverse clinical manifestations. While hepatic involvement, often presenting as mild biochemical abnormalities, is frequently observed, progression to end-stage liver disease (ESLD) is exceedingly rare. This case report describes an unusual case of SLE presenting with ESLD and highlights the importance of considering SLE in the differential diagnosis of cryptogenic liver diseases.

Materials and Methods. We report the case of a 47-year-old male who presented with fever, anemia, and signs of hepatic dysfunction. Laboratory investigations revealed severe anemia, thrombocytopenia, and hypergammaglobulinemia. Thoracoabdominal computed tomography showed bilateral pleural effusions, ascites, and a nodular liver with heterogeneous density, suggestive of chronic liver disease. Immunological tests confirmed positivity for antinuclear antibodies (ANA), anti-double-stranded DNA (anti-dsDNA), and anti-Ro/SSA antibodies. Markers for viral hepatitis and other autoimmune liver diseases were negative. A liver biopsy revealed porto-portal fibrosis with interface hepatitis, severe macrovesicular steatosis, and iron deposits, consistent with end-stage cirrhosis. The diagnosis of SLE was confirmed based on the 2019 EULAR/ACR classification criteria.

Results. The patient was diagnosed with Child-Pugh class B cirrhosis with a MELD score of 14. The combination of cytopenias, hypergammaglobulinemia, specific SLE autoantibodies, and the severe histopathological findings of the liver biopsy supported the diagnosis of SLE with significant hepatic involvement. Treatment with hydroxychloroquine and corticosteroids led to partial hematological improvement.

Conclusions. This case underscores the importance of considering SLE in the differential diagnosis of cryptogenic liver disease, especially in patients with positive autoimmune serologies. Early recognition and appropriate immunosuppressive therapy are crucial and may alter the disease course, potentially preventing progression to ESLD. Clinicians should be aware of the rare but severe hepatic manifestations of SLE.