Budd-Chiari syndrome secondary to antiphospholipid syndrome -: Clinical and immunologic characteristics of 43 patients

The clinical and immunologic characteristics of 43 patients with Budd-Chiari syndrome (BCS) and antiphospholipid syndrome (APS) (4 from our hospital and 39 from a computer-assisted review of the literature from 1983 through September 2000) are described. Twenty-nine (67%) patients were female and 14 (33%) male. Mean age at presentation of BCS was 30.8 ± 12.3 years. Thirty-two (74%) patients had primary APS, 8 (19%) had defined systemic lupus erythematosus, and the other 3 had APS associated with rheumatoid arthritis, lupus-like syndrome, and ulcerative colitis, respectively. In 28 (65%) patients, BCS was the first clinical manifestation of APS, whereas 9 (21%) patients had a previous history of major venous occlusion, arterial occlusion occurred in 1 (2%) patient, and spontaneous fetal losses had occurred in 10 (35%) of the 29 female patients. Thrombocytopenia was reported in 60% of patients, and hemolytic anemia was detected in 65%. LA was demonstrated in 77% of patients and the aCL titer was positive in 94% of patients. Most patients (80%) were positive for IgG isotype of aCL, whereas 59% were positive for the IgM isotype. Both LA and aCL positivity was detected in 63% of patients. Antinuclear antibodies were positive in 35% of patients, although usually in low titers. Anticoagulation was the most frequent treatment (84% of the patients), followed by steroids (37%), aspirin (11%), cyclophosphamide (8%), and plasmapheresis (3%). Different types of surgical derivative procedures were performed in 29% of patients, and percutaneous transluminal angioplasty in 11%. Our 4 patients were treated with a transjugular intrahepatic portosystemic shunt due to the presence of portal hypertension and progressive liver failure. Early dysfunction after the deployment of the stent was observed in 3 patients and was corrected with the insertion of a new coaxial stent and angioplasty. Of 9 patients who presented with vascular occlusions before the development of BCS, only 1 was on prolonged anticoagulant treatment. Of 7 patients who did not receive prolonged anticoagulant therapy after being diagnosed with BCS, 4 had new thrombotic events. Nineteen of 25 (76%) patients with prolonged anticoagulant therapy were in good health at a mean follow-up of 24 months (range, 1-96 mo). Six of the 31 (19%) patients with outcomes reported died, the causes of death being hepatic failure (2 patients), and massive gastrointestinal hemorrhage, Enterobacter septicemia, massive hemoptysis due to thrombocytopenia, and probably catastrophic APS (1 case each). The results of the present review stress the clinical importance of searching for the presence of LA and aCL in all patients with hepatic vein thrombosis. In these patients, a transjugular intrahepatic portosystemic shunt may be an effective treatment for portal hypertension, but in any case, long-term high-intensity anticoagulation is mandatory to prevent recurrent thrombosis.