Koch J W, al Nawaiseh I, Koch F H
Universitäts-Augenklinik Bonn.
Klin Monbl Augenheilkd. 1992 Nov;201(5):330-6. doi: 10.1055/s-2008-1045912.
Retinal vascular manifestation is the most common form of ophthalmic involvement in patients with systemic lupus erythematosus (SLE). Most frequently these consist of cotton-wool spots with or without intraretinal hemorrhages. Although rare, a more severe retinal vaso-occlusive disease, termed retinal vasculitis, has been described. We report on a 37-year-old white female with a 13-year follow-up of chronic discoid lupus erythematosus, which suffered massive bilateral visual loss coincident with the systemic exacerbation of her disease (proteinuria, pneumonia, serositis, leucopenia). The diagnosis of SLE was established with reference to the revised ARA-criteria (American Rheumatism Association). Ophthalmoscopy and fluorescein angiography revealed the typical aspect of a SLE-associated vaso-occlusive retinopathy on both eyes with marked ischemia of the macula. Immediate maximal immuno-suppressive therapy, early performed panretinal photocoagulation and subsequent cryoretinopexy did not stop the progression of the disease. Six months after the initial event vascularisations of the disc and rubeosis iridis occurred, but no secondary glaucoma up to date. In this patient, the almost complete absence of characteristic autoantibodies and immunological markers was striking. The correlation with other lupus manifestations, different therapeutic concepts and prognostic factors in SLE-associated retinal vasculitis are discussed.
视网膜血管表现是系统性红斑狼疮（SLE）患者眼部受累最常见的形式。最常见的是棉絮斑，可伴有或不伴有视网膜内出血。虽然罕见，但已描述了一种更严重的视网膜血管闭塞性疾病，称为视网膜血管炎。我们报告了一名37岁的白人女性，她患有慢性盘状红斑狼疮，经过13年的随访，在疾病全身加重（蛋白尿、肺炎、浆膜炎、白细胞减少）时出现双侧大量视力丧失。根据修订的ARA标准（美国风湿病协会）确诊为SLE。眼底检查和荧光素血管造影显示双眼典型的SLE相关血管闭塞性视网膜病变，黄斑明显缺血。立即进行最大程度的免疫抑制治疗、早期进行全视网膜光凝和随后的冷冻视网膜固定术均未能阻止疾病进展。初始事件发生6个月后，视盘血管化和虹膜红变出现，但至今未发生继发性青光眼。在该患者中，几乎完全缺乏特征性自身抗体和免疫标志物的情况令人瞩目。本文讨论了SLE相关视网膜血管炎与其他狼疮表现的相关性、不同的治疗理念和预后因素。
