[韦格纳肉芽肿的眼部表现]
[Ocular presentation in Wegener granulomatosis].
作者信息
Torres R M, Herreras J M, Becerra E, Blanco G, Méndez M C, Saornil M A
机构信息
Unidad de Superfice e Immunología Ocular, Instituto Universitario de Oftalmobiología Aplicada, Universidad de Valladolid, Valladolid, Spain.
出版信息
Arch Soc Esp Oftalmol. 2004 Mar;79(3):135-8. doi: 10.4321/s0365-66912004000300008.
CASE REPORT
A 71 year-old female presented with bilateral conjunctivitis, scleritis and peripheral ulcerative keratitis. Ancillary tests showed presence of anti-neutrophil cytoplasmic antibodies, with diffuse granular cytoplasmic immunofluorescence staining (ANCAc). Conjunctival biopsy showed necrotizing vasculitis in small vessels. Diagnosis of Wegener Granulomatosis (WG) was established and upper respiratory affection detected. Treatment with megadoses of cyclophosphamide and corticoids was initiated and remission of the disease was obtained.
DISCUSSION
WG is a potentially lethal systemic vasculitis in which ocular involvement may be the first clinical presentation. Scleritis and peripheral ulcerative keratitis indicate systemic involvement, highlighting the importance of prompt diagnosis and treatment.
病例报告
一名71岁女性出现双侧结膜炎、巩膜炎和周边溃疡性角膜炎。辅助检查显示存在抗中性粒细胞胞浆抗体，呈弥漫性颗粒状胞浆免疫荧光染色（c-ANCA）。结膜活检显示小血管坏死性血管炎。确诊为韦格纳肉芽肿（WG），并检测到上呼吸道受累。开始使用大剂量环磷酰胺和皮质类固醇进行治疗，疾病得以缓解。
讨论
韦格纳肉芽肿是一种潜在致命的系统性血管炎，眼部受累可能是其首发临床表现。巩膜炎和周边溃疡性角膜炎提示全身受累，凸显了及时诊断和治疗的重要性。
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