La Rosa A, Elourimi G, Zmuda M, Cucherousset N, Tran Ba S, Warzocha U, Larroche C, Sené T, Héran F, Galatoire O, Dhôte R, Abad S
Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Avicenne, Service de Médecine Interne, Bobigny, France.
Fondation Adolphe De Rothschild, Service de Chirurgie OculoPlastique, Paris, France.
Rev Med Interne. 2020 Dec;41(12):800-808. doi: 10.1016/j.revmed.2020.07.006. Epub 2020 Aug 27.
To describe a case series of patients investigated in internal medicine for orbital inflammation (OI) since the individualization of the clinical entity of the IgG4-related orbital disease (IgG4 ROD).
Thirty four patients were consecutively referred by a specialized center where orbital biopsy was performed when the lesion was surgically accessible. Fourteen patients were excluded in case of missing data or lymphoma, periocular xanthogranuloma or Graves' orbitopathy. Patients with systemic or auto-immune disease according to the international criteria, or presenting with idiopathic orbital inflammation syndrome (IOIS), were included. Knowing the histological similarities between IOIS and IgG4 ROD, immunohistochemical assessment of plasma cells for IgG4 positivity was performed for every patient with available biopsy. Clinical and biological characteristics, treatment and response to treatment of included patients are reported.
Among 22 included patients, 10 presented with orbital manifestation of a systemic or autoimmune disease including 2 sarcoidosis (9%) and 8 (36%) cases of non specific OI which were reclassified in IgG4 ROD. Finally, IOIS of various clinicopathological presentation was diagnosed for 12 patients including 10 with histological documentation. Whereas relapse and resistance were not found to be related to IgG4 positivity (50% in both IOIS and IgG4 ROD groups), another treatment in addition to corticosteroids was more often necessary in IgG4 ROD patients (50%) than in IOIS patients (25%).
After ruling out auto-immune orbital diseases, especially IgG4 ROD, IOIS should be discussed. Factors conditioning the corticosteroid response are yet to be determined.
描述自IgG4相关眼眶疾病（IgG4 ROD）临床实体个体化以来，在内科接受眼眶炎症（OI）检查的一系列病例。
34例患者由一个专业中心连续转诊，当病变可通过手术获取时，在该中心进行眼眶活检。14例患者因数据缺失或患有淋巴瘤、眼周黄色瘤或格雷夫斯眼病而被排除。纳入符合国际标准的全身性或自身免疫性疾病患者，或患有特发性眼眶炎症综合征（IOIS）的患者。鉴于IOIS与IgG4 ROD在组织学上存在相似性，对每例有可用活检样本的患者进行浆细胞IgG4阳性的免疫组织化学评估。报告纳入患者的临床和生物学特征、治疗及治疗反应。
在22例纳入患者中，10例表现为全身性或自身免疫性疾病的眼眶表现，包括2例结节病（9%）和8例（36%）非特异性OI，后者被重新分类为IgG4 ROD。最终，12例患者被诊断为各种临床病理表现的IOIS，其中10例有组织学记录。虽然未发现复发和耐药与IgG4阳性有关（IOIS组和IgG4 ROD组均为50%），但与IOIS患者（25%）相比，IgG4 ROD患者（50%）更常需要在使用皮质类固醇之外采用其他治疗。
在排除自身免疫性眼眶疾病，尤其是IgG4 ROD后，应讨论IOIS。影响皮质类固醇反应的因素尚待确定。
