Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Department of Hospital Pathology, College of Medicine, The Catholic University of Korea, Seoul, Korea.
Korean J Intern Med. 2019 Jan;34(1):220-226. doi: 10.3904/kjim.2016.304. Epub 2017 Oct 19.
BACKGROUND/AIMS: This study investigated the clinical and pathological features of immunoglobulin G4 (IgG4)-related ophthalmic disease. To clarify the features, we compared IgG4-related ophthalmic disease and orbital inflammatory pseudotumor.
We retrospectively reviewed the medical records of 103 patients who were initially diagnosed with orbital inflammatory pseudotumor, and identified 16 cases in which the diagnosis was based on surgical biopsy and for which data in medical records were sufficient for analysis. Immunohistochemical staining of pathological specimens for IgG and IgG4 was performed. Finally, six of IgG4-related ophthalmic disease patient and 10 of orbital inf lammatory pseudotumor patient were analyzed.
The IgG4-related ophthalmic disease group had more IgG4-positive plasma cells and a higher IgG4/IgG plasma cell ratio than the orbital inflammatory pseudotumor group. Collagenous fibrosis and lacrimal gland involvement were significantly more frequent in the IgG4-related ophthalmic disease group. Dense lymphocyte infiltration, obliterative phlebitis, and bilateral lesions were more frequent in IgG4-related ophthalmic disease, but the differences were not significant. The recurrence-free period was shorter in the IgG4-related ophthalmic disease group (p = 0.035).
The location of the lesion (lacrimal gland), count and ratio of IgG4-positive plasma cells, and collagenous fibrosis aid the diagnosis of IgG4-related ophthalmic disease in patients with idiopathic orbital mass-like lesions. In addition, maintenance therapy should be considered in patients with IgG4-related ophthalmic disease to prevent recurrence.
背景/目的：本研究调查了 IgG4 相关性眼病的临床和病理学特征。为了阐明其特征，我们比较了 IgG4 相关性眼病和眼眶炎性假瘤。
我们回顾性分析了 103 例最初诊断为眼眶炎性假瘤的患者的病历，并在其中确定了 16 例基于手术活检且病历中数据足以进行分析的病例。对病理标本进行 IgG 和 IgG4 的免疫组织化学染色。最后，分析了 6 例 IgG4 相关性眼病患者和 10 例眼眶炎性假瘤患者。
与眼眶炎性假瘤组相比，IgG4 相关性眼病组的 IgG4 阳性浆细胞更多，IgG4/IgG 浆细胞比值更高。胶原纤维纤维化和泪腺受累在 IgG4 相关性眼病组中更为常见。在 IgG4 相关性眼病组中，淋巴细胞密集浸润、闭塞性静脉炎和双侧病变更为常见，但差异无统计学意义。IgG4 相关性眼病组的无复发生存期较短（p=0.035）。
病变部位（泪腺）、IgG4 阳性浆细胞的数量和比值以及胶原纤维纤维化有助于诊断特发性眼眶肿块样病变患者的 IgG4 相关性眼病。此外，应考虑对 IgG4 相关性眼病患者进行维持治疗以预防复发。
