Abad S, Héran F, Terrada C, Bielefeld P, Sène D, Trad S, Saadoun D, Sève P
UMR1125, LI2P, service de médecine interne, hôpital Avicenne, faculté de médecine SMBH, université Paris 13, Sorbonne Paris Cité, Assistance publique-hôpitaux de Paris (AP-HP), 125, route de Stalingrad, 93000 Bobigny cedex 09, France.
Service d'imagerie, Fondation Adolphe-de-Rothschild, 25, rue Manin, 75019 Paris, France.
Rev Med Interne. 2018 Sep;39(9):746-754. doi: 10.1016/j.revmed.2017.12.010. Epub 2018 Feb 15.
Inflammatory orbitopathies relate to an inflammatory state originating within the orbit and its adnexes, except the inner ocular globe. Orbital inflammation (OI) may be either localized manifestation of a proven or like autoimmune disease, or local response from immune system against infectious, structural or tumoral antigens. We review the clinical manifestations of OI, which provide helpful clues to the diagnosis and describe the inflammatory, infectious and neoplastic conditions classically associated with OI. Autoimmune diseases are probably the most common causes of OI associated with a bilateral dacryoadenitis (e.g., sarcoidosis, granulomatosis with polyangiitis, IgG4-related disease). We focused on a major part of the IgG4-RD spectrum, the IgG4-related orbital disease which has been recently described and the idiopathic orbital inflammation syndrome that one should consider in patients 40 years of age or older with non specific inflammation OI on biopsy but without underlying local or systemic disease. An algorithm for the diagnostic approach of OI was proposed. If systemic explorations fail to diagnose an underlying disease, histopathologic control is required for distinguishing non-specific OI from other differential diagnosis, especially lymphoma. In the cases of pure myositic locations and posteriorly located tumours where biopsy could damage to the optic nerve, analysis of orbital lesions in T2W IRM sequence may be helpful to distinguish idiopathic OI (IOI) from lymphoma. When the diagnostic work-up fails, a corticosteroid trial could be used, but its beneficial effect has to be cautiously interpretated before definitively diagnosing IOI. Finally, treatments used in main infllammatory orbitopathies were also reviewed.
炎性眼眶病与起源于眼眶及其附件（眼球内部除外）的炎症状态有关。眼眶炎症（OI）可能是已证实的或疑似自身免疫性疾病的局部表现，也可能是免疫系统针对感染性、结构性或肿瘤性抗原的局部反应。我们回顾了OI的临床表现，这些表现为诊断提供了有用线索，并描述了与OI经典相关的炎症性、感染性和肿瘤性疾病。自身免疫性疾病可能是与双侧泪腺炎相关的OI的最常见原因（例如，结节病、肉芽肿性多血管炎、IgG4相关疾病）。我们重点关注了IgG4-RD谱系的一个主要部分，即最近描述的IgG4相关眼眶疾病以及40岁及以上患者出现非特异性炎症性OI且活检无潜在局部或全身疾病时应考虑的特发性眼眶炎症综合征。提出了一种OI的诊断方法算法。如果全身检查未能诊断出潜在疾病，则需要进行组织病理学检查以区分非特异性OI与其他鉴别诊断，尤其是淋巴瘤。在单纯肌炎部位和活检可能对视神经造成损伤的后部肿瘤病例中，T2加权IRM序列的眼眶病变分析可能有助于区分特发性OI（IOI）与淋巴瘤。当诊断检查失败时，可以进行皮质类固醇试验，但在明确诊断IOI之前，必须谨慎解释其有益效果。最后，还回顾了主要炎性眼眶病的治疗方法。
