Department of Ophthalmology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
BMC Ophthalmol. 2021 Dec 27;21(1):447. doi: 10.1186/s12886-021-02210-z.
To investigate the clinical manifestations of orbital involvement in a large cohort of Chinese patients with IgG4-related disease (IgG4-RD).
A total of 573 patients with IgG4-related disease were included. We described and compared the demographic, clinical, laboratory and histopathologic findings from 314 patients with IgG4-related ophthalmic disease (IgG4-ROD) and 259 with extra-ophthalmic IgG4-RD.
Male predominance was found significant in extra-ophthalmic IgG4-RD only. Patients with IgG4-ROD showed younger age at diagnosis and longer duration from onset till diagnosis. In patients with extra-ophthalmic IgG4-RD, the most commonly involved extra-ophthalmic organ was pancreas; while in IgG4-ROD patients, salivary gland was most frequently affected. Multivariate analysis exhibited IgG4-ROD was associated with allergy history, higher serum IgG4/IgG ratio, multiple organs involvement and sialoadenitis. Orbital images were reviewed in 173 (55.1%) IgG4-ROD patients. Fifty-one (29.5%) patients had multiple lesions. Lacrimal gland involvement was detected in 151 (87.3%) patients, followed by extraocular muscles (40, 23.1%), other orbital soft tissue (40, 23.1%) and trigeminal nerve (8, 4.6%). Biopsy was performed from various organs in 390 cases. A dense lymphoplasmacytic infiltration and fibrosis were the main feature in orbital specimens. Storiform fibrosis and obliterative phlebitis were absent in lacrimal gland.
Lacrimal gland involvement was the most common orbital manifestation of IgG4-ROD. Patients with IgG4-ROD showed different characteristic in demographic, clinical, laboratory findings compared to patients with extra-ophthalmic IgG4-RD. These features might indicate potential differences in the pathogenesis of these two subgroups of IgG4-RD.
研究 IgG4 相关疾病（IgG4-RD）中国患者大样本眼眶受累的临床表现。
共纳入 573 例 IgG4-RD 患者。我们描述并比较了 314 例 IgG4 相关眼病（IgG4-ROD）和 259 例眼外 IgG4-RD 患者的人口统计学、临床、实验室和组织病理学表现。
仅在外眼 IgG4-RD 中发现男性优势显著。IgG4-ROD 患者的诊断年龄更小，从发病到诊断的时间更长。在外眼 IgG4-RD 患者中，最常受累的眼外器官是胰腺；而在 IgG4-ROD 患者中，唾液腺最常受累。多变量分析显示 IgG4-ROD 与过敏史、血清 IgG4/IgG 比值升高、多器官受累和唾液腺炎有关。在 173 例（55.1%） IgG4-ROD 患者中回顾了眼眶图像。51 例（29.5%）患者有多个病变。151 例（87.3%）患者有泪腺受累，其次是眼外肌（40 例，23.1%）、其他眼眶软组织（40 例，23.1%）和三叉神经（8 例，4.6%）。对 390 例患者的各种器官进行了活检。眼眶标本的主要特征是致密的淋巴浆细胞浸润和纤维化。在泪腺中不存在席纹状纤维化和闭塞性静脉炎。
泪腺受累是 IgG4-ROD 最常见的眼眶表现。与眼外 IgG4-RD 患者相比，IgG4-ROD 患者在人口统计学、临床和实验室表现方面具有不同特征。这些特征可能表明这两个 IgG4-RD 亚组在发病机制上存在潜在差异。
