Duggal Lalit, Singh Bhandari Gurbir, Patel Jeet, Gupta Mayank, Grover Ashok K, Jain Neeraj
From the Departments of Rheumatology.
Ophthalmology, Sir Ganga Ram Hospital, New Delhi, India.
J Clin Rheumatol. 2022 Jan 1;28(1):e56-e62. doi: 10.1097/RHU.0000000000001591.
Immunoglobulin G4-related disease (IgG4-RD) is often an unrecognized, rare fibroinflammatory condition that can involve various organ systems. This study aimed to identify the different clinical patterns of this disease in a single center in North India.
Patients were diagnosed on the basis of published diagnostic criteria for IgG4-RD. Patients' presenting complaints; epidemiologic profiles; and laboratory, radiologic, and histologic findings along with the treatment and outcomes were collected and analyzed.
In total, 70 patients were diagnosed with the disease. The female-to-male ratio was 0.94:1, and it increased with multiorgan involvement. The mean age of patients was 41.4 years, and the majority of the patients (65.7%) were younger than 50 years. Patients were diagnosed as possible (38.57%), probable (32.85%), and definite (28.57%) IgG4-RD. The incidence of the involvement of orbital and periorbital tissues was the highest (52.9%); however, 13% of the patients had multiple organ involvement. Patients with involvement of the retroperitoneal tissues and the lymph nodes were 8.5% and 5.7%, respectively. Increased serum IgG4 levels were found in 74.3% of the patients with single-organ involvement, whereas all patients with multiorgan involvement had increased IgG4 levels. The majority of patients (94.3%) required immunosuppressive medications along with corticosteroids. Azathioprine was the most commonly used (72.8%) immunosuppressive medication. Rituximab was used in 17.1% of the patients, of whom only one had multisystem involvement.
This study depicts the most common patterns of organ involvement, along with the epidemiologic, laboratory, histologic, and radiologic data and response to treatment, in IgG4-RD, with a definite ophthalmology referral bias.
免疫球蛋白G4相关性疾病（IgG4-RD）通常是一种未被识别的罕见纤维炎症性疾病，可累及多个器官系统。本研究旨在确定印度北部一个单一中心该疾病的不同临床模式。
根据已发表的IgG4-RD诊断标准对患者进行诊断。收集并分析患者的主诉、流行病学资料、实验室、影像学和组织学检查结果以及治疗情况和预后。
共70例患者被诊断为此病。男女比例为0.94:1，且随着多器官受累而增加。患者的平均年龄为41.4岁，大多数患者（65.7%）年龄小于50岁。患者被诊断为可能（38.57%）、很可能（32.85%）和确诊（28.57%）的IgG4-RD。眼眶和眶周组织受累的发生率最高（52.9%）；然而，13%的患者有多个器官受累。腹膜后组织和淋巴结受累的患者分别为8.5%和5.7%。单器官受累患者中74.3%血清IgG4水平升高，而所有多器官受累患者的IgG4水平均升高。大多数患者（94.3%）需要免疫抑制药物联合皮质类固醇治疗。硫唑嘌呤是最常用的（72.8%）免疫抑制药物。17.1%的患者使用了利妥昔单抗，其中只有1例有多系统受累。
本研究描述了IgG4-RD中最常见的器官受累模式，以及流行病学、实验室、组织学和影像学数据及治疗反应，存在明确的眼科转诊偏向。
