Lai Kenneth K H, Li Emmy Y M, Chan Regine Y C, Wong Kenneth C W, Yu Jimmy K S, Cheuk W, Hui Y H, Cheng Andy C O, Chin Joyce K Y, Ip S K, Chan W H, Kwok Jeremy S W, Lam W C, Io Ida Y F, Mak Theresa S T, Li Kenneth K W, Lam N M, Yip Wilson W K, Young Alvin L, Chan Edwin, Ko Callie K L, Ko Simon T C, Yuen Hunter K L, Tham Clement C Y, Pang Chi Pui, Chong Kelvin K L
Department of Ophthalmology, 66380Tung Wah Eastern Hospital, Hong Kong.
71113Hong Kong Eye Hospital, Hong Kong.
Eur J Ophthalmol. 2023 Jan;33(1):171-181. doi: 10.1177/11206721221102274. Epub 2022 Jun 8.
Clinical phenotypes in Immunoglobulin G4-related disease (IgG4-RD) according to the patterns of affecting organs have different risks of malignancies. We attempt to determine the association of malignancies with IgG4-related ophthalmic disease (IgG4-ROD).
Retrospective cohort study.
Review of medical records, orbital images and histopathology reports in a territory-wide cohort of biopsy proven IgG4-ROD patients from 2005-2019.
Among 122 patients who had biopsies taken from adnexal lesions including lacrimal glands (n = 108), orbital mass (n = 30), infiltrated orbital fat (n = 10), conjunctiva (n = 2) or extraocular muscles (n = 3), 13% (16/122) developed malignancies over 73 ± 48months' follow-up. There were 9 cases of ocular adnexal lymphoma (OAL) and 7 extra-orbital malignancies. Compared with the general population, the incidence of OAL was significantly higher (standardized incidence ratios, SIRs = 10.0, 95%CI = 4.5-17.6) while that of extra-orbital malignancies was similar. The SIRs was highest within the first year (SIR = 46.7, 95%CI = 18.5-87.6) when 7 OAL were concomitantly diagnosed. Patients who developed OAL or extra-orbital malignancies were older than other patients at IgG4-ROD diagnosis (64.9 ± 7.1, 68.3 ± 8.5 versus 55.2 ± 15.0 years, P < 0.05). Asymmetric lacrimal gland enlargement (78% versus 13%), lack of frontal (0% versus 12%) or infraorbital nerve enlargement (0% versus 36%) were associated with OAL (all P < 0.05). Pre-treatment serum IgG4 level or extra-orbital IgG4-RD involvement was similar among patients with or without malignancies.
In this biopsy-proven IgG4-ROD cohort, 7% developed OAL which was 10 times higher than the general population. Patients with asymmetric lacrimal gland enlargement or without trigeminal nerves involvement radiologically were associated with OAL.
根据受累器官模式，免疫球蛋白G4相关疾病（IgG4-RD）的临床表型具有不同的恶性肿瘤风险。我们试图确定恶性肿瘤与IgG4相关眼病（IgG4-ROD）之间的关联。
回顾性队列研究。
回顾2005年至2019年在全地区范围内经活检证实的IgG4-ROD患者队列的病历、眼眶图像和组织病理学报告。
在122例接受附件病变活检的患者中，包括泪腺（n = 108）、眼眶肿物（n = 30）、眼眶脂肪浸润（n = 10）、结膜（n = 2）或眼外肌（n = 3），在73±48个月的随访中，13%（16/122）发生了恶性肿瘤。有9例眼附属器淋巴瘤（OAL）和7例眶外恶性肿瘤。与普通人群相比，OAL的发病率显著更高（标准化发病比，SIRs = 10.0，95%CI = 4.5-17.6），而眶外恶性肿瘤的发病率相似。在第一年SIRs最高（SIR = 46.7，95%CI = 18.5-87.6），当时同时诊断出7例OAL。发生OAL或眶外恶性肿瘤的患者在IgG4-ROD诊断时比其他患者年龄更大（64.9±7.1岁、68.3±8.5岁对55.2±15.0岁，P < 0.05）。泪腺不对称肿大（78%对13%）、无额神经（0%对12%）或眶下神经增粗（0%对36%）与OAL相关（均P < 0.05）。有或无恶性肿瘤患者的治疗前血清IgG4水平或眶外IgG4-RD受累情况相似。
在这个经活检证实的IgG4-ROD队列中，7%发生了OAL，比普通人群高10倍。泪腺不对称肿大或影像学上无三叉神经受累的患者与OAL相关。
