Ehongo A, Bremer F, Zanen A, Decaux G, Hanotier P, Kallay O
Service d'Ophtalmologie, Hôpital Erasme, U.L.B.
Bull Soc Belge Ophtalmol. 1996;263:9-14.
A man aged 68 years presents superior limbal infiltrates at his left eye two weeks before a marginal ulcer which quickly perforates. He has no systemic complaint. Clinical, biological, radiologic and histological evaluations disclose superior airways and lungs implications, an inflammatory syndrome, high ANCA (antineutrophiles cytoplamic antibodies) titer and vasculitis. There is no sign of renal involvement. A limited form of Wegener's granulomatosis is diagnosed. The outcome is favorable with a partial penetrating keratoplasty and systemic corticosteroid therapy in association with immunosuppressive drugs. This so called limited form of Wegener's granulomatosis is sight threatening when eye is the initial presentation. The early diagnostic and treatment will be performed by the help of ANCA in cases with subclinical systemic manifestations.
一名68岁男性在左眼出现角膜缘上部浸润两周后出现边缘性溃疡,并迅速穿孔。他没有全身不适症状。临床、生物学、放射学和组织学评估显示上呼吸道和肺部受累、炎症综合征、抗中性粒细胞胞浆抗体(ANCA)滴度高以及血管炎。没有肾脏受累的迹象。诊断为局限性韦格纳肉芽肿。通过部分穿透性角膜移植术以及全身使用皮质类固醇并联合免疫抑制药物治疗,预后良好。这种所谓的局限性韦格纳肉芽肿以眼部为首发表现时会威胁视力。对于有亚临床全身表现的病例,借助ANCA可进行早期诊断和治疗。