Nakamura H, Uehara H, Okada T, Kambe H, Kimura Y, Ito H, Hayashi E, Yamamoto H, Kishimoto S
Third Department of Internal Medicine, Osaka University Hospital, Japan.
Hepatogastroenterology. 1989 Oct;36(5):393-7.
We report the case of a woman with lupus anticoagulant-positive systemic lupus erythematosus who developed small hepatic vein occlusion. Since the age of 34, she had been known to have hepatomegaly. A definitive diagnosis of systematic lupus erythematosus was made eight years later. Histological evaluation of the liver biopsy specimen was not fully diagnostic of prominent hepatomegaly during this period. Occlusion of the small hepatic veins was confirmed by hepatic venography, but the lumen of the large hepatic veins showed a smooth appearance. The lupus anticoagulant and anti-cardiolipin antibody were both positive. Since a high incidence of thromboembolic diseases in patients with the lupus anticoagulant or anti-cardiolipin antibody has been reported, the presence of this type of anticoagulant may provide an explanation for hypercoagulability and subsequent development of hepatic vein thrombosis in this patient. This is the first report of a patient with systemic lupus erythematosus who developed an occlusion of small hepatic veins attributable to the lupus anticoagulant and anticardiolipin antibody. This case suggested that a systematic search for hepatic vein occlusion should be made in patients with systemic lupus erythematosus who have developed inexplicable hepatomegaly, especially in those with positive tests for the lupus anticoagulant and/or anti-cardiolipin antibody.
我们报告了一例狼疮抗凝物阳性的系统性红斑狼疮女性患者，该患者出现了小肝静脉闭塞。自34岁起，她就被发现有肝肿大。八年后确诊为系统性红斑狼疮。在此期间，肝活检标本的组织学评估并未完全诊断出明显的肝肿大。肝静脉造影证实了小肝静脉闭塞，但大肝静脉管腔外观光滑。狼疮抗凝物和抗心磷脂抗体均为阳性。由于已有报道称狼疮抗凝物或抗心磷脂抗体阳性的患者血栓栓塞性疾病的发生率较高，这种抗凝物的存在可能解释了该患者的高凝状态以及随后发生的肝静脉血栓形成。这是首例系统性红斑狼疮患者因狼疮抗凝物和抗心磷脂抗体导致小肝静脉闭塞的报告。该病例提示，对于出现无法解释的肝肿大的系统性红斑狼疮患者，尤其是狼疮抗凝物和/或抗心磷脂抗体检测呈阳性的患者，应进行系统性的肝静脉闭塞检查。
