Hernanz I, Larque A B, Quintana L F, Espigol-Frigole G, Espinosa G, Adan A, Sainz-de-la-Maza M
Clinical Institute of Ophthalmology, Hospital Clinic of Barcelona, Barcelona, Spain.
Department of Pathology, Hospital Clinic of Barcelona, Barcelona, Spain.
Am J Ophthalmol Case Rep. 2021 Apr 22;22:101100. doi: 10.1016/j.ajoc.2021.101100. eCollection 2021 Jun.
To describe a case series of scleritis associated with IgA vasculitis (IgAV) at a tertiary referral center.
Three men with scleritis associated with IgAV were identified: one with anterior scleritis alone, one with anterior scleritis and peripheral ulcerative keratitis (sclerokeratitis), and one with anterior and posterior scleritis. Visual acuity was preserved except from the patient who developed posterior scleritis. Ocular pain was the main symptom at presentation. All patients had a previous history of palpable purpura, but only one was aware of his underlying IgAV. Laboratory results revealed microhematuria and proteinuria with normal urinary β microglobulin levels and negative serum ANCAs. Skin or kidney biopsy demonstrated leukocytoclastic vasculitis or glomerulonephritis with dominant IgA immune deposits.
Although uncommon, IgAV should be included in the differential diagnosis of anterior scleritis alone or associated with peripheral ulcerative keratitis or posterior scleritis, even in systemically asymptomatic patients. Urinalysis should not be underestimated in assessment of scleritis to detect early stages of glomerular disease. Scleritis may be the first manifestation whose study may lead to the diagnosis of IgAV. Multidisciplinary approach is necessary to prevent irreversible organ damage such as renal failure.
描述一家三级转诊中心一系列与IgA血管炎（IgAV）相关的巩膜炎病例。
确定了3例与IgAV相关的巩膜炎男性患者：1例仅患有前巩膜炎，1例患有前巩膜炎和周边溃疡性角膜炎（巩膜角膜炎），1例患有前、后巩膜炎。除了发生后巩膜炎的患者外，其他患者的视力均得以保留。眼部疼痛是就诊时的主要症状。所有患者既往均有可触及的紫癜病史，但只有1例意识到自己潜在的IgAV。实验室检查结果显示镜下血尿和蛋白尿，尿β微球蛋白水平正常，血清抗中性粒细胞胞浆抗体（ANCA）阴性。皮肤或肾脏活检显示白细胞破碎性血管炎或肾小球肾炎，伴有主要的IgA免疫沉积物。
尽管IgAV并不常见，但即使在全身无症状的患者中，对于单独的前巩膜炎或合并周边溃疡性角膜炎或后巩膜炎的情况，鉴别诊断时也应考虑IgAV。在评估巩膜炎以检测肾小球疾病早期阶段时，不应低估尿液分析。巩膜炎可能是首发表现，对其进行研究可能会导致IgAV的诊断。多学科方法对于预防诸如肾衰竭等不可逆的器官损伤是必要的。
