Department of Rheumatology, Hospital de Pediatría Centro Médico Nacional Siglo XXI, IMSS, Av. Cuauhtémoc # 330, Col. Doctores, CP 06720, Mexico City, Mexico.
Department of Pediatrics, Hospital Gabriel Mancera IMSS, Mexico City, Mexico.
Rheumatol Int. 2024 Nov;44(11):2679-2690. doi: 10.1007/s00296-023-05366-7. Epub 2023 Jun 15.
Idiopathic orbital inflammation (IOI) is a diagnosis of exclusion, but the exclusion of other inflammatory diseases of the orbit is broad and relies on clinician experience, response to corticosteroid, or biopsy. This study aimed to investigate the presence of granulomatosis with polyangiitis (GPA) in patients initially diagnosed as IOI and describe its clinicopathological features, ANCA status, treatment, and outcome. We performed a retrospective case series study of children diagnosed with limited GPA (L-GPA) in patients with IOI. A systematic review of the literature was performed in children with GPA and orbital mass. Eleven of 13 (85%) patients with IOI had L-GPA. Two additional patients with orbital mass and L-GPA were included in this analysis. The median age was 10 years, and 75% were female. Twelve cases were ANCA positive and 77% were MPO-pANCA positive. Most patients had a poor response to treatment and had a high relapse rate. Based on literature review, 28 cases were found. Most (78.6%) were female with a median age of 9 years. Three patients were misdiagnosed as IOI. Patients with L-GPA more frequently had MPO-pANCA positivity (35%) than children with systemic GPA (18%) and were less often PR3-cANCA positive than patients with systemic GPA (18% vs. 46%). L-GPA accounts for a high prevalence of children diagnosed as IOI. The high prevalence of MPO-pANCA observed in our study may be related to L-GPA rather than with the orbital mass. Long-term follow-up, orbital biopsy, and serial ANCA testing are necessary to exclude GPA in patients with IOI.
特发性眼眶炎症（IOI）是一种排除性诊断，但排除其他眼眶炎症性疾病的范围很广，依赖于临床医生的经验、对皮质类固醇的反应或活检。本研究旨在调查最初诊断为 IOI 的患者中是否存在肉芽肿性多血管炎（GPA），并描述其临床病理特征、ANCA 状态、治疗和预后。我们对诊断为 IOI 的儿童进行了 GPA 局限型（L-GPA）的回顾性病例系列研究。对儿童 GPA 和眼眶肿块进行了文献系统回顾。13 例 IOI 患者中有 11 例（85%）患有 L-GPA。本分析还纳入了另外 2 例患有眼眶肿块和 L-GPA 的患者。中位年龄为 10 岁，75%为女性。12 例为 ANCA 阳性，77%为 MPO-pANCA 阳性。大多数患者对治疗反应不佳，复发率高。根据文献复习，发现 28 例。大多数（78.6%）为女性，中位年龄为 9 岁。有 3 例患者误诊为 IOI。与系统性 GPA 患儿（18%）相比，L-GPA 患儿更常出现 MPO-pANCA 阳性（35%），而与系统性 GPA 患儿相比，PR3-cANCA 阳性率较低（18% vs. 46%）。L-GPA 在被诊断为 IOI 的儿童中占很高的比例。在我们的研究中观察到的 MPO-pANCA 高患病率可能与 L-GPA 有关，而与眼眶肿块无关。对 IOI 患者进行长期随访、眼眶活检和连续 ANCA 检测对于排除 GPA 是必要的。
