Jindal B K, Martin M F, Gayner A
Ann Rheum Dis. 1983 Jun;42(3):347-9. doi: 10.1136/ard.42.3.347.
We report a case of progressive peripheral ischaemia and gangrene as a presenting feature of systemic lupus erythematosus. It developed in a previously asymptomatic 40-year-old woman following minor surgery to her toe. Eventually she required a below-knee amputation and despite systemic corticosteroids continued to deteriorate, presenting later with signs of systemic intravascular thromboses. Histopathology and immunofluorescence on vessels repeatedly failed to demonstrate any evidence for vasculitis. A full coagulation screen confirmed the presence of 'lupus' anticoagulant. A plasma exchange was performed to remove circulating immunoglobins and she made a rapid and sustained recovery. Peripheral gangrene has not previously been described in association with lupus anticoagulant. We would suggest that in all cases of systemic thrombosis or unexplained peripheral vascular ischaemia lupus anticoagulant should be considered.
我们报告一例以进行性外周缺血和坏疽为特征的系统性红斑狼疮病例。该病例发生在一名40岁既往无症状的女性身上，起因是她的脚趾接受了小手术。最终，她需要进行膝下截肢，尽管使用了全身性皮质类固醇药物，但病情仍持续恶化，随后出现了系统性血管内血栓形成的迹象。对血管进行的组织病理学和免疫荧光检查反复未能显示任何血管炎的证据。全面的凝血筛查证实存在“狼疮”抗凝物。进行了血浆置换以清除循环免疫球蛋白，她迅速并持续康复。此前尚未有外周坏疽与狼疮抗凝物相关的描述。我们建议，在所有系统性血栓形成或不明原因外周血管缺血的病例中，都应考虑狼疮抗凝物。
