Stevens J M, Hunt J E, Seymour A E, Krilis S A, Pugsley D J
Renal Unit, Queen Elizabeth Hospital, Woodville, South Australia.
Clin Nephrol. 1994 Jan;41(1):18-22.
Sneddon's syndrome, cerebrovascular thrombosis and livedo reticularis, is often a variant of the "primary" anti-phospholipid syndrome (PAPS). We report a woman with PAPS, presenting as Sneddon's syndrome, with renal impairment and glomerular thrombosis on renal biopsy. An IgG anti-cardiolipin antibody (aCL) was identified. The aCL was purified by affinity chromatography, gel filtration chromatography and ion-exchange chromatography, assayed in a modified ELISA and found to be of the type that requires the plasma protein beta 2-GPI to bind aCL. As beta 2-GPI has anticoagulant properties it is postulated that its interaction with aCL has a pathogenic role in the thrombotic lesions associated with aCL.
斯内登综合征，即脑血管血栓形成与网状青斑，通常是“原发性”抗磷脂综合征（PAPS）的一种变异型。我们报告一例患有PAPS的女性，表现为斯内登综合征，肾活检显示有肾功能损害和肾小球血栓形成。检测到一种IgG抗心磷脂抗体（aCL）。通过亲和层析、凝胶过滤层析和离子交换层析对aCL进行纯化，在改良酶联免疫吸附测定（ELISA）中进行检测，发现其属于需要血浆蛋白β2-糖蛋白I（β2-GPI）来结合aCL的类型。由于β2-GPI具有抗凝特性，因此推测其与aCL的相互作用在与aCL相关的血栓形成病变中具有致病作用。
