31例IgG4相关性疾病的临床分析

doi:10.13701/j.cnki.kqyxyj.2021.02.018

摘要/Abstract

摘要： 目的:提高对IgG4相关性疾病(IgG4-related disease,IgG4-RD)的认识及诊治水平。方法:分析31例就诊于新疆医科大学第一附属医院诊断为IgG4-RD患者的临床表现、影像学、血清学、病理学、治疗等特点,并使用IgG4-RD应答者指数评估疾病活动和损害。结果:31例患者中,男18例,女13例,平均年龄(59.1±9.12)岁,起病到诊断的中位时间为10(1~120)个月。首诊误诊6例,误诊率为19.35%。32.26%的患者合并过敏性疾病。影像学主要表现为2个及2个以上受累器官增大和(或)占位,主要以唾液腺为主(77.42%),其次是淋巴结、泪腺、胆道等。IgG4-RD应答者指数为7.13±3.25,与受累器官呈正相关(r=0.15,P<0.0001)。93.55%的患者血清IgG4升高,且与血清IgG呈正相关(r=0.54,P<0.01)。病理检查可见淋巴浆细胞浸润,淋巴滤泡增生明显,组织间伴不规则纤维化。25例服用糖皮质激素患者中,1例因治疗无效改用利妥昔单抗治疗,2例联合免疫抑制剂治疗,随访时间为(25.89±14.02)个月。12例患者治疗后效果良好,复查IgG4水平下降,12例患者在治疗期间出现复发现象,复发率为48%。1例治疗期间因突发心脏病死亡。结论:IgG4-RD是一种常见于中老年男性的多系统纤维炎症性疾病。唾液腺、淋巴结、泪腺及内脏器官肿大是常见的临床表现,病理检查是确诊的金标准,糖皮质激素及生物制剂对其治疗有效,但存在复发现象。

关键词: IgG4相关性疾病, 临床特点, 诊断, 治疗

Abstract: Objective: To improve the understanding, diagnosis, and treatment of IgG4-RD. Methods: The clinical, imaging, serological, pathological, and therapeutic features of 31 patients with IgG4-RD diagnosed in the first affiliated Hospital of Xinjiang Medical University were analyzed, and IgG4-RD responder index was used to evaluate disease activity and damage. Results: Among the 31 patients, there were 18 males and 13 females, with an average age of (59.1±9.12) years. The median time from onset to diagnosis was 10 months (range 1-120 months). Six cases were misdiagnosed at the first diagnosis, with a misdiagnosis rate of 19.35%. 32.26% of the patients were complicated with allergic diseases. Imaging revealed that all patients had organ occupying, and all patients had 2 or more organs involved, mainly salivary gland (77.42%), followed by lymph nodes, lacrimal gland, bile duct, and so on. The IgG4-RD responder index was 7.13±3.25, and it was positively correlated with the involved organs (r=0.15, P<0.0001). Serum IgG4 increased in 93.55% of these patients, and it was positively correlated with serum IgG (r=0.54, P<0.01). Histopathology showed infiltration of lymphatic plasma cells, obvious proliferation of lymphoid follicles and irregular fibrosis between tissues. Among the 25 patients who took glucocorticoid drugs, one patient was treated with rituximab because of ineffective treatment, and two patients were treated with immunosuppressants. The follow-up time was (25.89±14.02) months. Twelve patients had good results after treatment, the level of IgG4 decreased. Twelve patients had recurrence during treatment, with a recurrence rate of 48%. One case died of heart attack during treatment. Conclusion: IgG4-RD is a multi-system fibro-inflammatory disease, which is common in middle-aged and elderly men. Enlargement of salivary glands, lymph nodes, lacrimal glands, and internal organs is a common clinical manifestation. Glucocorticoids and biological agents are effective in the treatment, but there is a recurrence.

Key words: IgG4-related diseases, clinical characteristics, diagnosis, treatment

蒲娇, 胡露露, 牛雅琪, 贾梦莹, 曾雪敏, 何伟, 龚忠诚. 31例IgG4相关性疾病的临床分析[J]. 口腔医学研究, 2021, 37(2): 173-178.

PU Jiao, HU Lulu, NIU Yaqi, JIA Mengying, ZENG Xuemin, HE Wei, GONG Zhongcheng. Clinical Analysis of Thirty-one Cases of IgG4-related Disease[J]. Journal of Oral Science Research, 2021, 37(2): 173-178.

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