National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Internal Medicine, Hospital Universitario 12 de Octubre, Instituto de Investigación Hospital 12 de Octubre (i+12), School of Medicine, Universidad Complutense, Madrid, Spain.
National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Internal Medicine, Institut Mutualiste Montsouris, Paris, France.
Autoimmun Rev. 2019 May;18(5):493-500. doi: 10.1016/j.autrev.2019.03.001. Epub 2019 Mar 4.
To analyse the ocular manifestations of patients with GPA, their treatment and outcome.
Retrospective cohort study performed at the National Referral Center for Vasculitis, Cochin Hospital, Paris (France), from January 2005 to December 2015. Among 308 patients with a new diagnosis of GPA in accordance with the American College of Rheumatology classification criteria and/or revised Chapel Hill nomenclature definitions, we identified those with ocular involvement and a subsequent follow up in our center.
The prevalence of ocular involvement in our GPA series was 38.6%; 63 patients were analysed with a median follow-up of 50.5 months (IQR: 17.8-82.8). Scleritis (18 patients, 28.6%) and episcleritis (18 patients, 28.6%) were the most common ophthalmologic manifestations, followed by orbital disease (13 patients, 20.6%). Bilateral involvement and visual acuity loss was seen in 29.1% and 16.7% of patients, respectively. Ocular involvement was the first GPA manifestation in 9 patients (14.3%), concomitant with systemic manifestation in 36 (57.1%), and occurred only during follow-up in 18 (28.6%). The indication for GPA treatment was suggested by ocular involvement in 12 patients (19.0%), by systemic features in 40 (63.5%) and by both ocular and systemic involvement in 11 (17.5%). Remission of ocular involvement was achieved in 51 patients (80.9%). In the remaining 12 (19.1%), symptoms persisted or even worsened, finally leading to rituximab (RTX) therapy in 8 of them (66.7%). Altogether, when used as first line or for refractory disease, ocular remission was achieved in 11 of the 12 cases (91.7%) treated with RTX versus 34 of the 47 cases (72.3%) treated with CYC (P = .260). Eye disease relapsed in 14 patients (22.2%). RTX allowed achievement of remission in 8 of them (57.1%). In the remaining six, other immunosuppressive drugs were used.
Scleritis and episcleritis are the most common ocular manifestations in GPA, most of the time associated with other systemic manifestations. In >40% of cases, ocular manifestations were refractory to initial treatment or recurrent and led to RTX prescription, which appeared to be useful in these situations.
分析 GPA 患者的眼部表现、治疗方法和转归。
回顾性队列研究，在法国巴黎 Cochin 医院国家血管炎转诊中心进行，纳入 2005 年 1 月至 2015 年 12 月间符合美国风湿病学会分类标准和/或修订的 Chapel Hill 命名法定义的 308 例 GPA 新诊断患者，对这些患者进行眼部受累和后续随访。
在我们的 GPA 系列中，眼部受累的患病率为 38.6%；对 63 例患者进行了分析，中位随访时间为 50.5 个月（IQR：17.8-82.8）。巩膜炎（18 例，28.6%）和表层巩膜炎（18 例，28.6%）是最常见的眼部表现，其次是眶疾病（13 例，20.6%）。29.1%和 16.7%的患者分别出现双侧受累和视力丧失。9 例（14.3%）患者的眼部受累是 GPA 的首发表现，36 例（57.1%）患者伴有全身表现，18 例（28.6%）患者仅在随访中出现眼部受累。12 例（19.0%）患者因眼部受累而开始 GPA 治疗，40 例（63.5%）患者因全身表现而开始治疗，11 例（17.5%）患者因眼部和全身表现均开始治疗。51 例（80.9%）患者眼部受累缓解。在其余 12 例（19.1%）患者中，症状持续存在甚至恶化，最终其中 8 例（66.7%）接受利妥昔单抗（RTX）治疗。RTX 治疗共 12 例（100%），其中 11 例（91.7%）为初治或难治性疾病，而环磷酰胺（CYC）治疗共 47 例（91.7%），其中 34 例（72.3%）（P=0.260）。14 例（22.2%）患者眼部疾病复发。RTX 使 8 例（57.1%）患者的病情得到缓解。其余 6 例患者使用了其他免疫抑制剂。
巩膜炎和表层巩膜炎是 GPA 最常见的眼部表现，多数情况下与其他全身表现相关。>40%的患者对初始治疗或复发反应不佳，最终需要使用 RTX，RTX 在这些情况下似乎是有效的。
