Kleinknecht D, Bobrie G, Meyer O, Noël L H, Callard P, Ramdane M
Service de Néphrologie et Réanimation Polyvalente, Centre Hospitalier, Montreuik, France.
Nephrol Dial Transplant. 1989;4(10):854-8. doi: 10.1093/ndt/4.10.854.
In five patients suffering from recurrent thrombosis and/or fetal death, a lupus anticoagulant was associated with a renal vasculopathy. Ischaemic episodes also involved the skin, heart, eyes and/or central nervous system. All patients were hypertensive. Two had renal insufficiency, two had non-nephrotic proteinuria, and in the last patient renal cortical ischaemia was detected by a tomographic scan in the absence of proteinuria. Renal biopsy showed thrombosis and/or intimal fibrosis of intrarenal vessels, and normal or ischaemic glomeruli without proliferative lesions. High-titres of anticardiolipin antibodies were found in 3 of 3 cases, and persisted after steroid therapy even if the circulating anticoagulant factor disappeared. All patients received corticosteroid therapy, alone or in combination with immunosuppressive drugs; two patients had prolonged oral anticoagulation, but thrombotic episodes recurred after stopping the drug. One patient died; the remaining four survived 18 months to 11 years after diagnosis, with stable chronic renal insufficiency in one of them. These results show that a lupus anticoagulant may be associated with prominent renal vascular disease, in the absence of proliferative glomerular lesions, and suggest that continuous anticoagulation may be beneficial in these patients.
在5例复发性血栓形成和/或胎儿死亡患者中，狼疮抗凝物与肾血管病相关。缺血发作还累及皮肤、心脏、眼睛和/或中枢神经系统。所有患者均患有高血压。2例患者有肾功能不全，2例患者有非肾病性蛋白尿，最后1例患者在无蛋白尿的情况下经断层扫描发现肾皮质缺血。肾活检显示肾内血管血栓形成和/或内膜纤维化，肾小球正常或缺血，无增殖性病变。3例患者中有3例检测到高滴度抗心磷脂抗体，即使循环抗凝因子消失，在类固醇治疗后抗体仍持续存在。所有患者均接受了皮质类固醇治疗，单独或联合免疫抑制药物；2例患者接受了长期口服抗凝治疗，但停药后血栓形成事件复发。1例患者死亡；其余4例在诊断后存活18个月至11年，其中1例患有稳定的慢性肾功能不全。这些结果表明，在无增殖性肾小球病变的情况下，狼疮抗凝物可能与显著的肾血管疾病相关，并提示持续抗凝可能对这些患者有益。
