Vasculitis Center, Oskar-Alexander-Str. 26, 24576 Bad Bramstedt, Germany.
Rheumatology (Oxford). 2013 May;52(5):875-82. doi: 10.1093/rheumatology/kes382. Epub 2013 Jan 4.
To identify and characterize patients with orbital masses in a monocentric cohort of 1142 GPA patients followed up from 1990 until the end of 2010 with regard to disease stage, local orbital inflammation, course of disease and outcome and to assess the efficacy of immunosuppressive treatment.
All GPA patients fulfilling ACR criteria or Chapel Hill Consensus Conference definitions or who had localized GPA and who developed orbital masses were evaluated regarding the course and outcome of the orbital masses (assessed by MRI, ophthalmologist and ENT specialist), all other clinical manifestations, disease stages, ANCA status, immunosuppression and its side effects and surgical procedures.
Of 1142 GPA patients 58 developed orbital masses during a median follow-up of 101.5 months (range 23-255 months). Forty patients fulfilled the inclusion criteria and had complete clinical assessments [44% females, median age 43 (20-74) years, 85% ANCA positive]. Seventy-five per cent (29/40) had systemic disease when orbital masses occurred; both orbits were affected in 30%. Seventy-two per cent had evidence of infiltration from paranasal sinuses. Under highly potent immunosuppression (mostly CYC and glucocorticoids), 41% were refractory, 24% had unchanged activity, 24% showed a response and 8.1% had complete remission. Forty-four per cent had relapses of orbital masses. Seventy-two per cent developed visual impairment, 19% suffered blindness. Blindness was associated with a longer time to remission and a relapsing and refractory course.
Orbital masses are a rare manifestation of GPA and are characterized by a refractory course and by a high rate of local damage. Patients with a refractory or relapsing course are at higher risk of developing blindness.
通过对 1990 年至 2010 年底期间的 1142 例 GPA 患者进行回顾性分析，确定并描述这些患者的眼眶肿块特征，分析其疾病分期、局部眼眶炎症、病程和预后，并评估免疫抑制治疗的效果。
所有满足 ACR 标准或 Chapel Hill 共识会议定义的 GPA 患者，或患有局限性 GPA 且发生眼眶肿块的患者，均接受了眼眶肿块（通过 MRI、眼科医生和耳鼻喉科医生评估）、所有其他临床表现、疾病分期、抗中性粒细胞胞浆抗体 (ANCA) 状态、免疫抑制及其副作用和手术程序的评估。
在 1142 例 GPA 患者中，58 例在中位随访 101.5 个月（23-255 个月）期间发生眼眶肿块。40 例患者符合纳入标准且具有完整的临床评估[44%为女性，中位年龄 43（20-74）岁，85%的患者 ANCA 阳性]。当眼眶肿块发生时，75%（29/40）的患者有系统性疾病；30%的患者累及双侧眼眶。72%的患者有来自副鼻窦的浸润证据。在高强度免疫抑制（主要为 CYC 和糖皮质激素）下，41%的患者为难治性，24%的患者病情无变化，24%的患者有反应，8.1%的患者完全缓解。44%的患者出现眼眶肿块复发。72%的患者出现视力障碍，19%的患者失明。失明与缓解时间延长和复发难治性病程有关。
眼眶肿块是 GPA 的一种罕见表现，其特征为难治性病程和高局部损害率。病程难治或复发的患者发生失明的风险更高。
