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Abstract：
Background/Objectives: IgG4-related ophthalmic disease (IgG4-ROD), characterized by lymphoplasmacytic infiltration, fibrosis, and elevated IgG4 levels, presents diagnostic challenges while offering insights into immune-mediated inflammatory disorders. The aim of this study was to comprehensively examine the clinical features and outcomes of IgG4-ROD. Materials and Methods: A retrospective study was conducted on 33 patients diagnosed with IgG4-ROD, fulfilling the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria. The demographic characteristics of the IgG4-ROD patients were compared with those of 37 patients diagnosed with IgG4-related disease (IgG4-RD) in departments other than ophthalmology (IgG4-nonROD) at the same hospital during the same period. The patients diagnosed with IgG4-ROD were initially treated with glucocorticosteroid (GCS) monotherapy, GCS combined with steroid-sparing agents (SSAs; mycophenolate mofetil, azathioprine, hydroxychloroquine), biologic agent (rituximab) monotherapy, or watchful waiting. The primary outcome was the assessed treatment response at 6 months, and the secondary outcome was the evaluation of recurrence at 1 year after initial treatment. A response was evaluated as the absence of ocular signs and symptoms, either clinically or radiologically. Results: Eyelid swelling (17 patients, 51.5%) was the most common symptom, and lacrimal gland (17 patients, 51.5%) was the most frequent site of involvement. The response rate for GCS monotherapy was 33.3% (3 out of 9 patients), while the response rate for GCS combined with SSA was 60.0% (9 out of 15 patients). The lacrimal gland group demonstrated a significantly higher treatment response compared to the non-lacrimal gland group (66.7% vs. 20.0%, p = 0.013), and the combination of GCS and SSA resulted in a significantly higher treatment response than the GCS monotherapy (77.8% vs. 33.3%, p = 0.045). The group including hydroxychloroquine (HCQ), which comprised 5 out of 33 patients (15.2%), showed no recurrence at 1 year. Conclusions: The combination therapy of GCS and SSA for IgG4-ROD can be considered an effective treatment approach and HCQ could be considered as a potential adjunctive therapy for IgG4-ROD.
摘要：
背景/目标：IgG4相关眼科疾病（IgG4-ROD），以淋巴浆细胞浸润为特征，纤维化，和升高的IgG4水平，提出了诊断挑战，同时提供了对免疫介导的炎症性疾病的见解。这项研究的目的是全面检查IgG4-ROD的临床特征和结果。材料与方法：对33例诊断为IgG4-ROD的患者进行回顾性研究。符合美国风湿病学会/欧洲抗风湿病联盟（ACR/EULAR）标准。在同一时期，将IgG4-ROD患者的人口统计学特征与在同一医院眼科以外的其他科室（IgG4-nonROD）诊断为IgG4相关疾病（IgG4-RD）的37例患者进行了比较。诊断为IgG4-ROD的患者最初接受糖皮质激素（GCS）单药治疗，GCS联合类固醇保护剂（SSAs；霉酚酸酯，硫唑嘌呤，羟氯喹)，生物制剂（利妥昔单抗）单一疗法，或警惕的等待。主要结果是6个月时评估的治疗反应，次要结局是评估初次治疗后1年的复发.反应被评估为没有眼部体征和症状，临床或放射学。结果：眼睑肿胀（17例，51.5%)是最常见的症状，和泪腺（17例，51.5%)是最常见的参与地点。GCS单药治疗的反应率为33.3％（9例患者中有3例），而GCS联合SSA的缓解率为60.0%(15例患者中有9例)。与非泪腺组相比，泪腺组表现出明显更高的治疗反应（66.7％vs.20.0%,p=0.013),与GCS单一疗法相比，GCS和SSA的组合产生了显着更高的治疗反应（77.8％vs.33.3%,p=0.045)。该组包括羟氯喹（HCQ），33名患者中有5名(15.2%)，1年无复发。结论：GCS和SSA联合治疗IgG4-ROD可被认为是一种有效的治疗方法，HCQ可被认为是IgG4-ROD的潜在辅助治疗方法。
