Outcome and prognosis of Budd Chiari syndrome patients with Antiphospholipid syndrome

Abstract

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 According to duration of symptoms and signs of liver disease, BCS can be presented in acute, subacute or chronic form; the most common presentation is the chronic form. A high index of suspicion is necessary for diagnosis because clinical manifestations and laboratory results are non specific. The goals of treatment are to prevent extension of thrombosis in the hepatic veins and to alleviate venous obstruction in order to decrease hepatic congestion. Few patients respond to medical treatment (anticoagulation with or without thrombolytic therapy, diuretics). However, most of patients need more invasive procedures to restore the hepatic blood flow including percutaneous angioplasty with or without stenting, transjugular intrahepatic portosystemic shunt (TIPS) or shunt surgery. Antiphospholipid syndrome (APS) is a disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss. Characteristic laboratory abnormalities in APS include persistently elevated levels of antibodies directed against membrane anionic phospholipids (ie, anticardiolipin [aCL] antibody, antiphosphatidylserine) or their associated plasma proteins, predominantly beta-2 glycoprotein I (apolipoprotein H); or evidence of a circulating anticoagulant. 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 Research criteria for defining the APS. Adapted from Clinical Criteria 1. Vascular thrombosis: One or more clinical episodes of arterial, venous or small vessel thrombosis

2. Pregnancy morbidity: (a) One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation (b) One or more pre-term births of a morphologically normal neonate before the 34th week of gestation because of: (i) eclampsia or severe pre-eclampsia or (ii) recognized features of placental insufficiency (c) Three or more unexplained consecutive spontaneous miscarriages before the 10th week of gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded Laboratory criteria 1. Lupus anticoagulant (LA) present in plasma, on two or more occasions at least 12 weeks apart 2. Anticardiolipin (aCL) antibody of immunoglobulin IgG and/or IgM isotype in serum or plasma, present in medium or high titer (i.e.>40GPL units or MPL units, or>the 99th centile), on two or more occasions, at least 12 weeks apart 3. Anti-b2–glycoprotein I antibody of IgG and/or IgM isotype in serum or plasma (in titre>the 99th centile), present on two or more occasions at least 12 weeks apart Antiphospholipid antibody syndrome (APS) is present if at least one of the clinical criteria and one of the laboratory criteria are met.