Garrity J A, Kennerdell J S, Johnson B L, Ellis L D
Am J Ophthalmol. 1986 Jul 15;102(1):97-103. doi: 10.1016/0002-9394(86)90217-5.
When vasculitis, an angiocentric and angiodestructive process, occurs in the orbit, the clinical presentation and radiographic findings resemble those of idiopathic inflammatory pseudotumor. Three patients, two men and a woman, 28 to 72 years old, initially thought to have "pseudotumor" failed to response to corticosteroid therapy. Orbital biopsy specimens in all patients disclosed vasculitis. There was no evidence of systemic vasculitis. High-dose prednisone effectively eliminated pain and reduced inflammation but did not adequately control fibrosis formation leading to ultimate loss of function. Each patient eventually lost an eye to this process. Therapy with cyclophosphamide, a B-cell cytotoxic drug, produced a prompt response in terms of eliminating pain, inflammation, and formation of fibrous tissue. Cyclophosphamide therapy has been instrumental in preserving sight in each patient's remaining eye. In such cases we believe the benefits of cyclophosphamide therapy outweight the known risks.
血管炎是一种以血管为中心并具有血管破坏性的病变过程，当它发生于眼眶时，其临床表现和影像学表现与特发性炎性假瘤相似。3例患者，2男1女，年龄28至72岁，最初被认为患有“假瘤”，但对皮质类固醇治疗无反应。所有患者的眼眶活检标本均显示为血管炎。无全身血管炎的证据。大剂量泼尼松有效消除了疼痛并减轻了炎症，但未能充分控制纤维化形成，最终导致功能丧失。每个患者最终都因这个过程而失去了一只眼睛。使用B细胞细胞毒性药物环磷酰胺进行治疗，在消除疼痛、炎症和纤维组织形成方面产生了迅速的反应。环磷酰胺治疗有助于保护每位患者另一只眼睛的视力。在这种情况下，我们认为环磷酰胺治疗的益处超过了已知的风险。
