Unveiling a Rare Association: Budd-Chiari Syndrome as a Consequence of Antiphospholipid Antibody Syndrome and Systemic Lupus Erythematosus

Disha Patel, Darsh Patel, Prachi Parikh, Parth Patel

Abstract: Budd-Chiari syndrome, arising from the obstruction of hepatic venous outflow, poses a significant medical challenge due to its varied causes. One infrequent cause is its link to Systemic Lupus Erythematosus (SLE) in conjunction with Anti-Phospholipid Antibody Syndrome (APLA), an autoimmune disorder characterized by abnormal blood clotting. We present a case report of an 18-year-old male who presented with abdominal distension, jaundice, and splenomegaly. Diagnostic investigations revealed hepatic vein thrombosis, consistent with Bud-Chiari syndrome, and further workup confirmed APLA associated with SLE. This rare association emphasizes the importance of considering antiphospholipid antibodies and SLE in young patients presenting with Bud-Chiari syndrome, even in the absence of typical SLE manifestations. Timely identification and early anticoagulation therapy play a crucial role in preventing potentially devastating complications of venous thrombosis in this context.

Keywords: Budd-Chiari Syndrome, Systemic Lupus Erythematosus, Anti-Phospholipid Antibody Syndrome