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

E Dhivya,Gitanjali Dasari,S Selvamani,V R Mohan Rao

doi:10.9734/jpri/2021/v33i47b33090

E Dhivya, Gitanjali Dasari + Show 2 more

Open Access

https://doi.org/10.9734/jpri/2021/v33i47b33090

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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.

Highlights

Autoimmune disorders like Systemic Lupus Erythematosus (SLE) usually presents with fatiguability, malar rash, fever, arthralgia, photosensitivity, alopecia but rarely presents with Budd-Chiari syndrome stemming from the root cause of Antiphospholipid antibody syndrome (APLA)
Antiphospholipid syndrome is characterised by the production of autoantibodies directed against phospholipids and phospholipid binding plasma proteins associated with multiple thromboembolic events
We report a young female who presented with abdominal distension, abdominal pain and fever diagnosed to have Budd-Chiari syndrome

Summary

INTRODUCTION

Autoimmune disorders like Systemic Lupus Erythematosus (SLE) usually presents with fatiguability, malar rash, fever, arthralgia, photosensitivity, alopecia but rarely presents with Budd-Chiari syndrome stemming from the root cause of Antiphospholipid antibody syndrome (APLA). The common manifestations are recurrent foetal loss followed by portal vein thrombosis, mesenteric thrombosis, iliofemoral thrombosis and lastly Budd-Chiari syndrome [2]. SLE presenting with Budd-Chiari syndrome as an early manifestation is unusual and rarely reported in the literature. We report a young female who presented with abdominal distension, abdominal pain and fever diagnosed to have Budd-Chiari syndrome. On further evaluation, she was found to have antiphospholipid syndrome secondary to SLE

CASE REPORT

CONCLUSION