Objective: To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China. Methods: IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016. All patients were followed-up for more than 6 months. The demographic characteristics, symptoms, organ involvements, laboratory examinations and treatment efficacy were evaluated and analyzed. Results: A total of 346 patients were finally enrolled, including 230 males (66.5%) and 116 females (33.5%). The mean age of disease onset was (53.8±14.2) years old. The mostly common involved organs were lymph nodes (56.4%) and submandibular glands (52.6%). Other affected organs and manifestations included: swelling of the lacrimal glands (46.5%), autoimmune pancreatitis (38.4%), pulmonary involvement (28.0%), sclerosing cholangitis (25.4%), naso-sinusitis (23.4%), parotid gland swelling (21.7%), retroperitoneal fibrosis (19.9%), large arteries involvement (9.5%), kidney involvement (obstructive nephropathy caused by retroperitoneal fibrosis was excluded) (6.9%), skin lesions (6.4%). Rare features consisted of thyroid glands, pituitary glands, gastrointestinal tract, pachymeningitis, pericardium, sclerosing mediastinitis and orchitis. The majority of patients had multi-organ involvement, such as 74.3% patients with 3 and more, 18.2% and 7.5% patients with 2 and single organ involvement respectively. The average IgG4-RD responder index (IgG4-RD RI) was 13.21±5.70. History of allergy was found in 172 (49.7%) patients. As to the laboratory tests, elevated serum IgG4 levels were confirmed in 285 (94.1%) patients, which was positively correlated with IgG4-RD RI. There were 33.5% patients receiving monotherapy of glucocorticoid, 52.6% treated with glucocorticoids combined with immunosuppressive agents, 4.9% patients with immunosuppressant only, and 9.0% patients with mild disease not receiving medication. The majority (336, 97.1%) patients improved the above regimens. Conclusion: IgG4-RD is a systemic fibro-inflammatory disease with multiple organ involvement. The mostly common involved organs include lymph node, submandibular glands, and pancreas. Glucocorticoids and immunosuppressive agents were effective for IgG4-RD.
目的： 通过分析346例IgG4相关性疾病(IgG4－RD)患者的临床特征，旨在提高对该病的诊治水平。 方法： 选2011年1月—2016年1月纳入北京协和医院IgG4－RD前瞻性队列研究且随诊6个月以上的患者。分析患者人口统计学特征、受累器官特点、实验室检查及治疗情况。 结果： 346例IgG4－RD患者中男230例(66.5%)，女116例(33.5%)，发病年龄(53.8±14.2)岁。最常见的临床表现为淋巴结肿大195例(56.4%)和颌下腺肿大182例(52.6%)。其他器官受累依次为泪腺肿大161例(46.5%)，自身免疫性胰腺炎133例(38.4%)，肺97例(28.0%)，硬化性胆管炎88例(25.4%)，鼻窦81例(23.4%)，腮腺肿大75例(21.7%)，腹膜后纤维化69例(19.9%)，大动脉33例(9.5%)，肾脏24例(6.9%)，皮肤22例(6.4%)，少见受累包括甲状腺、垂体、胃肠道、硬脑膜/硬脊膜、心包、颅内占位、硬化性纵隔炎和睾丸。多数患者为多器官受累，257例(74.3%)患者受累器官≥3个，63例(18.2%)患者2个器官受累，26例(7.5%)患者单个器官受累。IgG4－RD反应指数为13.21±5.70。172例(49.7%)患者有过敏史。285例(94.1%)初治患者血清IgG4升高[8 320(3 764，18 600)mg/L]，且与IgG4－RD反应指数呈正相关(r＝0.430，P<0.001)。初始治疗时116例(33.5%)患者单用糖皮质激素，182例(52.6%)患者糖皮质激素联合免疫抑制剂，17例(4.9%)患者单用免疫抑制剂，31例(9.0%)患者未予药物治疗。336例(97.1%)患者病情改善或稳定。 结论： IgG4－RD是一种多器官受累的慢性炎症伴纤维硬化性疾病，最常受累器官是淋巴结、颌下腺、泪腺及胰腺。糖皮质激素和免疫抑制剂治疗IgG4－RD有效。.
Keywords: Clinical characteristics; Cohort study; IgG(4) related disease.