Department of Ophthalmology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Orbit. 2022 Oct;41(5):551-557. doi: 10.1080/01676830.2021.1962365. Epub 2021 Aug 9.
To analyse the incidence and characteristics of ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) with immunoglobulin G4 (IgG4)-related ophthalmic disease (IgG4-ROD), and to compare with IgG4-negative OAML.
We enrolled 130 patients with pathology-confirmed OAML at a single tertiary medical centre. Patients were divided into IgG4-positive and IgG4-negative groups based on the pathologic criteria for IgG4-ROD. The data were reviewed and compared between the groups.
IgG4-positive OAML was identified in 5.4% (7/130) of OAML and 13.5% (7/52) of non-conjunctival OAML. IgG4-positive group had a higher incidence of involvement of the lacrimal gland (7/7 vs. 18/123, = .001), extraocular muscles (3/7 vs. 10/123, = .022) and infraorbital nerve (2/7 vs. 0/123, < .001), and had a lower incidence of involvement of the conjunctiva (0/7 vs. 84/123; < .001) than IgG4-negative group. IgG4-positive group had higher T and N categories of the American Joint Committee on Cancer classification (T1:T2:T3:T4 ratio of 0:6:0:1 vs. 78:32:8:5, < .001; and ≥N1 ratio of 2/7 vs. 7/123, = .021). There were no differences in the response rate to initial treatment (5/6 vs. 39/41, = .343) or the relapse rate (1/6 vs. 5/41, = 1.000) between the groups.
IgG4-positive OAML showed clinical features similar to IgG4-ROD, such as predominant disease involvement of the lacrimal gland, extraocular muscles, infraorbital nerve and lymph nodes, but not the conjunctiva. However, treatment outcomes were favourable with or without background IgG4-ROD. Physicians should be aware that IgG4-ROD might undergo a malignant transformation and that thorough treatment and surveillance of IgG4-ROD are important.
分析伴有 IgG4 相关眼病（IgG4-ROD）的眼附属器黏膜相关淋巴组织淋巴瘤（OAML）的发病率和特征，并与 IgG4 阴性 OAML 进行比较。
我们在一家三级医疗中心纳入了 130 例经病理证实的 OAML 患者。根据 IgG4-ROD 的病理标准，将患者分为 IgG4 阳性和 IgG4 阴性两组。对两组患者的数据进行了回顾和比较。
在 130 例 OAML 患者中，5.4%（7/130）为 IgG4 阳性 OAML，在 52 例非结膜 OAML 中，13.5%（7/52）为 IgG4 阳性 OAML。IgG4 阳性组泪腺（7/7 比 18/123， = .001）、眼外肌（3/7 比 10/123， = .022）和眶下神经（2/7 比 0/123， < .001）受累的发生率较高，而结膜受累（0/7 比 84/123； < .001）的发生率较低。IgG4 阳性组美国癌症联合委员会（AJCC）分期的 T 和 N 类别更高（T1：T2：T3：T4 比为 0：6：0：1 比 78：32：8：5， < .001；N1 以上比为 2/7 比 7/123， = .021）。两组患者初始治疗的反应率（5/6 比 39/41， = .343）或复发率（1/6 比 5/41， = 1.000）均无差异。
IgG4 阳性 OAML 表现出与 IgG4-ROD 相似的临床特征，如主要累及泪腺、眼外肌、眶下神经和淋巴结，但不累及结膜。然而，有或没有背景 IgG4-ROD 的治疗结果均良好。医生应该意识到 IgG4-ROD 可能会发生恶性转化，彻底治疗和监测 IgG4-ROD 非常重要。
