A Case of Budd-Chiari Syndrome – As an Unusual Early Manifestation of Systemic Lupus Erythematosis with Antiphospholipid Syndrome and Deep Vein Thrombosis

Abstract

Budd-Chiari syndrome occurs due to obstruction of hepatic venous out-flow which in turn produces intense congestion of the liver. Systemic Lupus Erythematosus (SLE), which is an auto-immune disorder has protean manifestations such as easy fatiguability,  arthralgia, photosensitivity, malar rash, fever, alopecia but our case had an unique  association of  ascites and hepatosplenomegaly due to Budd Chiari syndrome stemming from the root cause of Antiphospholipid antibody syndrome (APLA) is very rare. SLE accounts for 40% of the cases of APLA. SLE presenting with Budd-Chiari syndrome as an early manifestation is unusual and is rarely reported in the literature. Here we report a rare case of a young female who presented with abdominal distension, abdominal pain and fever diagnosed to have Budd-Chiari syndrome as a presenting feature of SLE and on further evaluation she was also found to have  antiphospholipid syndrome secondary to SLE.