Objectives:
@EN Many patients with Budd-Chiari syndrome have no evident etiological factor especially in Asian countries. And various obstructive patterns of inferior vena cava and hepatic veins have been reported suggesting several different causes may be
involved. Recently primary antiphospholipid antibody syndrome has been described as a characteristic clinical entity with multiple thromboembolic episodes and typical laboratory features such as serum antiphospholipid antibody, not being
associated
with
any collagen vascular disease. To evaluate the etiological role of primary antiphospholipid antibody syndrome in Budd-Chiari syndrome and clarify the clinical features of Budd-Chiari syndrome patients with primary antiphospholipid antibody
syndrome, we
analyzed clinical and angiographic data of 27 consecutive patients with Budd-Chiari syndrome (Age:47±12 years, M: F=13:14).
@ES Methods:
@EN We analyzed clinical manifestations and angiographic characteristics of 4 Budd- Chiari syndrome patients with primary antiphospholipid antibody syndrome, comparing to those of 23 with out it.
@ES Results:
@EN Underlying etiological factors were identified only in 6(22%); 4(15%) were associated with primary antiphospholipid antibody syndrome.
Most of patients with Budd-Chiari syndrome showed superficial abdominal collaterals, ascites, symmetrical lower leg edema and hepatosplenomegaly with laboratory features of liver cirrhosis, regardless the association of primary antiphospholipid
antibody
syndrome. However, only out of 4 with primary antiphospholipid antibody syndrome, 2 had asymmetrical-lower leg edema with ulcer; 2 complained of unexplained long-standing dry cough, 1 of intermittent fever. In both with lower leg ulcer,
thrombotic
obstructions of deep veins were identified. Another one with primary antiphospholipid antibody syndrome was proved to have pulmonary hypertension without definite vascular obstruction. All of 4 patients(100%) with primary antiphospholipid
antibody
syndrome in contrast to only 8 out of 23(35%) without it showed broad obstruction of inferior vena cava and all three hepatic veins(Sugiura type II; p<0.05).
@ES Conclusion:
@EN These data suggested that primary antiphospholipid antibody syndrome is one of common etiological factors in patients with Budd-Chiari syndrome, and that especially in Budd-Chiari syndrome patients who present asymmetrical lower leg edema
with
ulcer, long-standing dry cough, unexplained fever, pulmonary hypertension of unknown cause or broad obstruction of inferior vena cava, the possibility of association with primary antiphospholipid antibody syndrome should be considered.
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