Prevalence of Antiphospholipid Syndrome in Primary Budd Chiari patients and their intervention outcome

Abstract Background Budd-Chiari syndrome (BCS) is a rare but potentially life threatening hepatic disorder that results from obstruction of the hepatic venous outflow tract. Obstruction can occur at any level from the hepatic venules to the right atrium. According to the etiology, BCS can be classified as primary (due to intrinsic intraluminal thrombosis or webs) or secondary (due to intraluminal invasion by a parasite or malignant tumor or extraluminal compression by an abscess, cyst or solid tumor) the clinical presentation of BCS has a wide spectrum and ranges from asymptomatic cases to fulminant hepatic failure The classic triad of abdominal pain, ascites, and hepatomegaly is nonspecific. Aim of the Work To assess the prevelance of Antiphospholipid syndrome (APS) in primary Budd Chiari syndrome (BCS) patients and their management outcome. Patients and Methods Patients presented to BCS outpatient clinic, Tropical medicine department, Ain Shams University Hospitals were enrolled in the study from 2005 to 2020. Some patients with APS have no evidence of any definable associated disease, while, in other patients, APS occurs in association with SLE or another rheumatic or autoimmune disorder. Traditionally, these have been referred to as primary or secondary APS, respectively. Results Regarding laboratory data, Lupus Anticoagulant, ACL IgG antibodies and ACL IgM antibodies were positive in 60 (83.3%), 49 (68.1%) and 27 (37.5%) patients respectively. For 42 patients (58.3%), intervention was performed. TIPSS was performed on 33 (45.8%) patients, while Angioplasty with Stent was performed on 9 (12.5%) patients. There was no intervention in 30 (41.7%) of the patients. In the studied patients, rate of mortality of the total 72 patients was 48.6%. The mean cause of death was Hepatorenal syndrome (31.4%). Conclusion BCS, although infrequent, is not a rare complication in patients with APS and may be the first clinical manifestation of this syndrome. However, no predisposing factor to BCS has been identified in patients with APS. This supports the need for systematic screening for LA and aCL in all cases of hepatic vein thrombosis. In these patients, TIPS placement may be an effective treatment for portal hypertension, but in any case, long-term high intensity anticoagulation treatment is essential in order to prevent recurrent thrombosis. Lupus anticoagulant is much more common than anticardiolipin antibodies (83.3%, 68.1% respectively) among BCS patients due to antiphospholipid syndrome.