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日本全国性免疫球蛋白G4相关疾病合并恶性肿瘤的流行病学调查。

Nationwide epidemiological survey of immunoglobulin G4-related disease with malignancy in Japan.

作者信息

Sumimoto Kimi, Uchida Kazushige, Ikeura Tsukasa, Hirano Kenji, Yamamoto Motohisa, Takahashi Hiroki, Nishino Takayoshi, Mizushima Ichiro, Kawano Mitsuhiro, Kamisawa Terumi, Saeki Takako, Maguchi Hiroyuki, Ushijima Tomoyuki, Shiokawa Masahiro, Seno Hiroshi, Goto Hiroshi, Nakamura Seiji, Okazaki Kazuichi

机构信息

The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Osaka, Japan.

Department of Gastroenterology and Hepatology, Kochi Medical School, Kochi University, Nankoku, Japan.

出版信息

J Gastroenterol Hepatol. 2022 Jun;37(6):1022-1033. doi: 10.1111/jgh.15809. Epub 2022 Mar 20.

DOI:10.1111/jgh.15809
PMID:35229347
Abstract

BACKGROUND AND AIM

To clarify the clinicoepidemiological characteristics of immunoglobulin G4 (IgG4)-related disease (IgG4-RD) with malignancy, a nationwide epidemiological survey was conducted.

METHODS

Immunoglobulin G4-related disease patients with malignancy who had visited selected hospitals in Japan were surveyed. The study consisted of two stages: the number of IgG4-RD patients with malignancy was estimated by the first questionnaire and their clinicoepidemiological characteristics were assessed by the second questionnaire.

RESULTS

The frequencies of autoimmune pancreatitis (AIP), IgG4-related sialadenitis, IgG4-related eye disease, IgG4-related kidney disease, and IgG4-related retroperitoneal fibrosis were 44.7%, 20.8%, 14.0%, 5.16%, and 5.12%, respectively. The overall prevalence of malignant disease in IgG4-RD cases was estimated to be 10 900 per 100 000 cases, which was significantly higher than that of malignant disease in the general population. The prevalence of malignant lymphoma in IgG4-RD cases was the highest and was estimated to be 1985 per 100 000 cases. IgG4-related kidney disease had the highest frequency of malignant disease (17.1%). In data from 200 patients, 61 (30.5%) cases of cancer were found 2 years or more before the IgG4-RD diagnosis, 92 cases (46%) during the 1 year preceding or following IgG4-RD diagnosis, and 62 cases of cancer (31%) 2 or more years following IgG4-RD diagnosis.

CONCLUSIONS

The nationwide survey for IgG4-RD with malignancy in Japan showed that IgG4-RD may be related with malignant diseases.

摘要

背景与目的

为阐明合并恶性肿瘤的免疫球蛋白G4(IgG4)相关疾病(IgG4-RD)的临床流行病学特征,开展了一项全国性的流行病学调查。

方法

对就诊于日本选定医院的合并恶性肿瘤的IgG4相关疾病患者进行调查。该研究包括两个阶段:通过第一份问卷估计合并恶性肿瘤的IgG4-RD患者数量,并通过第二份问卷评估其临床流行病学特征。

结果

自身免疫性胰腺炎(AIP)、IgG4相关涎腺炎、IgG4相关眼病、IgG4相关肾病和IgG4相关腹膜后纤维化的发生率分别为44.7%、20.8%、14.0%、5.16%和5.12%。IgG4-RD病例中恶性疾病的总体患病率估计为每10万例中有10900例,显著高于普通人群中的恶性疾病患病率。IgG4-RD病例中恶性淋巴瘤的患病率最高,估计为每10万例中有1985例。IgG4相关肾病的恶性疾病发生率最高(17.1%)。在200例患者的数据中,61例(30.5%)癌症病例在IgG4-RD诊断前2年或更早被发现,92例(46%)在IgG4-RD诊断前1年或诊断后1年内发现,62例(31%)癌症病例在IgG4-RD诊断后2年或更长时间发现。

结论

日本针对合并恶性肿瘤的IgG4-RD进行的全国性调查表明,IgG4-RD可能与恶性疾病有关。

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