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

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.


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). Budd-Chiari syndrome is caused by obstruction of hepatic venous outflow which produces intense congestion of the liver [1]. In Budd-Chiari syndrome the smaller hepatic veins are often spared while involving all the three major hepatic veins. Antiphospholipid syndrome is characterised by the production of autoantibodies directed against phospholipids and phospholipid binding plasma proteins associated with multiple thromboembolic events. The common manifestations are recurrent foetal loss followed by portal vein thrombosis, mesenteric thrombosis, iliofemoral thrombosis and lastly Budd-Chiari syndrome [2]. SLE accounts for 40% of the cases of APLA. SLE presenting with Budd-Chiari syndrome as an early manifestation is unusual and rarely reported in the literature. Here, 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
A 21 year old married female with no known comorbidities, presented with abdominal distension which was progressive over the period of 2 weeks which was associated with dull aching poorly localized abdominal pain. Thyroid function test were normal.COVID-19 RTPCR, blood cultures and sputum for AFB were negative. USG abdomen showed hepatomegaly with liver size of 19cm with early chronic liver disease, moderate ascites, splenomegaly with normal status of the kidneys. CECT abdomen showed thrombosis of the right hepatic vein (Fig-1

DISCUSSION
Budd-Chiari syndrome occurs due to the obstruction of hepatic venous out-flow which inturn produces intense congestion of the liver. Smaller hepatic veins are often spared in buddchiari syndrome. Polycythemia rubra vera, aspergillosis , filariasis, amoebic liver abscess, schistosomiasis, membranous obstruction of IVC, adrenal adenoma, Hepatocellular carcinoma, renal cell carcinoma, antiphospholipid syndrome, leiomyosarcoma of IVC, pregnancy, HRT, oral contraceptives are some of the causes of budd-chiari syndrome.
Clinically patients may have abdominal pain, abdominal distension, weakness, anorexia, jaundice, abdominal venous distension, massive ascites, hepatomegaly, splenomegaly and oedema of thighs, legs and feet. Antiphospholipid syndrome is an autoimmune disorder characterised by the formation of auto-antibodies against the phospholipids leading to the occurrence of multiple thrombotic events [3].
Another rheumatic, autoimmune and multisytemic disorder is systemic lupus erythematosus characterised by the presence of auto-antibodies. The association of SLE with Antiphospholipid Syndrome (APS) is about 36% in reported cases [4].
The association of antiphospholipid antibody syndrome (APLA) and SLE with Budd-Chiari syndrome is very rare and only few cases have been reported in literature. Most of the cases reported till now had clinical manifestations of SLE before they were diagnosed with Budd-Chiari. Our patient presented with Budd-chiari which on further work up was found to have SLE Espinosa G et al., [5] Jayabal et al., and Ilkgül O et al., also provided the data with the similar findings regarding the association of Budd-chiari syndrome as an early manifestation of SLE.. Patient with secondary antiphospholipid syndrome should be kept on long term antiplatelet or anticoagulants therapy to maintain the target INR between 3 to 4 [6]. Side to side porto-caval shunting can be done if there is inadequate response to medical therapy and in case of shunt failure [7], [8], Ortho topic liver transplantation can be considered. This is because of the thrombosis of the portal vein, splenic vein, and superior mesenteric vein which results in unshuntable portal hypertension.

CONCLUSION
Any patient presenting with features of Buddchiari syndrome, should always be screened for Antiphospholipid syndrome as well as for SLE even in the absence of the other clinical features prior to anticoagulation therapy and steroids especially in young females [9]. Early screening and diagnosis will prevent the future complications like repeated miscarriages and other multi-systemic complications.

CONSENT AND ETHICAL APPROVAL
As per international standard or university standard guideline Patient's consent and ethical approval has been collected and preserved by the authors.