Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients
Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients
复制标题
免疫球蛋白 G4 相关疾病的临床特征：118 名中国患者的前瞻性研究。
DOI：
10.1093/rheumatology/kev203
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发表时间：
2015-11-01
期刊：
影响因子：
5.5
通讯作者：
Lipsky, Peter E.
中科院分区：
文献类型：
--
作者：
Lin, Wei;Lu, Sha;Lipsky, Peter E.
Objective.目的了解我国IgG 4相关疾病（IgG 4-RD）的临床特点。2011年至2013年在北京协和医院进行了一项IgG 4-RD的前瞻性队列研究。入选新诊断的IgG 4-RD患者。共纳入118例IgG 4-RD患者，其中男性82例，女性36例，年龄53.1岁（S. D. 13.6年）。最常见的症状为泪腺肿胀（38/32.2%）。涉及一系列器官：77例患者淋巴结肿大65.3%，64.4%有涎腺炎，60泪腺炎50.8%，自身免疫性胰腺炎38.1%，肺部受累占27.1%，（26.3%）有腹膜炎/腹膜后纤维化，29例（35.4%）有前列腺炎，29例（24.6%）有肾脏受累。硬化性胆管炎21例（17.8%），鼻窦炎15例（12.7%），炎性假瘤10例（8.5%）。少见的表现包括纵隔纤维化、皮肤受累、硬化性甲状腺炎、垂体炎、睾丸炎和结肠炎。在93例患者中观察到多个器官受累，而仅4.2%的患者仅累及单个器官。73例（61.9%）患者报告了过敏史。97.5%的患者血清IgG_4水平升高，且与受累器官数相关。大多数患者接受糖皮质激素单用或联合免疫抑制剂治疗，大多数患者通常在3个月内好转。IgG 4-RD是一种全身性炎性硬化性疾病。腮腺和泪腺受累（以前称为Mikulicz病），淋巴结病和胰腺炎是最常见的表现。IgG 4-RD患者对糖皮质激素和免疫抑制剂的治疗反应良好。
Objective. To characterize the clinical features of IgG4-related disease (IgG4-RD) in China.Methods. A prospective cohort study of IgG4-RD was carried out in Peking Union Medical College Hospital between 2011 and 2013. Patients with newly diagnosed IgG4-RD were enrolled.Results. A total of 118 patients with IgG4-RD were enrolled, including 82 males and 36 females, aged 53.1 (S. D. 13.6) years. The most common symptom at onset was lacrimal gland swelling (38/32.2%). A range of organs were involved: 77 patients (65.3%) had lymphadenopathy, 76 (64.4%) had sialadenitis, 60 (50.8%) had dacryoadenitis, 45 (38.1%) had autoimmune pancreatitis, 32 (27.1%) had pulmonary involvement, 31 (26.3%) had periaortitis/retroperitoneal fibrosis, 29 (35.4% of male patients) had prostatitis and 29 (24.6%) had renal involvement. In addition, there were 21 (17.8%) cases of sclerosing cholangitis, 15 (12.7%) of sinusitis and 10 (8.5%) of inflammatory pseudotumour. Uncommon manifestations included mediastinal fibrosis, skin involvement, sclerosing thyroiditis, hypophysitis, orchitis and colitis. Multiple organ involvement was observed in 93 patients, whereas only 4.2% had only a single organ involved. A history of allergy was reported in 73 (61.9%) patients. The serum IgG4 level was elevated in 97.5% and was correlated with the number of organs involved. Most patients were treated with glucocorticoids alone or in combination with immunosuppressive drugs, and the majority usually improved within 3 months.Conclusion. IgG4-RD is a systemic inflammatory and sclerosing disease. Parotid and lacrimal involvement (formerly called Mikulicz's disease), lymphadenopathy and pancreatitis are the most common manifestations. Patients with IgG4-RD showed favourable responses to treatment with glucocorticoids and immunosuppressive agents.