New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases.
New clues to the nature of immunoglobulin G4-related disease: a retrospective Japanese multicenter study of baseline clinical features of 334 cases.
复制标题
DOI：
10.1186/s13075-017-1467-x
复制
发表时间：
2017-12-01
影响因子：
4.9
通讯作者：
Kawano M
中科院分区：
文献类型：
--
作者：
Yamada K;Yamamoto M;Saeki T;Mizushima I;Matsui S;Fujisawa Y;Hara S;Takahashi H;Nomura H;Kawa S;Kawano M
目的是通过由日本多学科医生对免疫球蛋白G4相关疾病(IgG4-RD)的临床和实验室特征进行的大规模多中心研究，进一步确定其特征。多位专家通过分析五家医院1996至2015年间确诊的IgG4-RD患者的基线临床特征、实验室、影像和病理检查结果以及治疗情况，对他们进行了回顾性评估。在列出的334名患者中，205名为男性，确诊时的中位年龄为65岁。确诊时受累的平均器官数为3.2个。最常见的受累器官是唾液腺，其次是泪腺、淋巴结、胰腺、腹膜后/主动脉周围、肾脏和肺。血清IgG4平均水平为755 mg/dl，超过95%的患者血清IgG4水平升高。血清C-反应蛋白(CRP)水平中位数为0.1 mg/dl，90%患者低于1 mg/dl。共有34.7%的患者血清C3水平较低。肾脏病变患者血清C3和非Ig G4抗体水平(总Ig G减去Ig G4)呈负相关，而血清Ig G4水平与血清C3水平无相关性。78.0%的患者使用了皮质类固醇，所有患者都有效。与西方患者相比，大多数日本IgG4-RD患者的血清CRP水平普遍较低，而血清IgG4水平较高。这些原始发现表明，IgG4-RD中的这两个参数在某些有趣的方面与迄今在西方人群中报道的不同。需要进行更多的研究，特别是国际比较研究，以阐明人口之间这些差异的程度和意义。还必须注意，在制定国际分类标准时，是否需要考虑这种差异的存在。本文的在线版本(doi：10.1186/s130750171467x)包含补充材料，授权用户可以使用。
The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan. Various specialists retrospectively evaluated IgG4-RD patients diagnosed between 1996 and 2015 in five hospitals by analyzing their baseline clinical features, laboratory, imaging, and pathological test findings, and treatment. Of the 334 patients listed, 205 were male and median age at diagnosis was 65 years. The mean number of organs involved was 3.2 at diagnosis. The most frequently affected organs were the salivary glands, followed by the lacrimal glands, lymph nodes, pancreas, retroperitoneum/periaorta, kidneys, and lungs. The mean serum level of IgG4 was 755 mg/dl, and more than 95% of patients had elevated serum IgG4 levels. The median serum level of C-reactive protein (CRP) was 0.1 mg/dl and the level was less than 1 mg/dl in 90% of patients. A total of 34.7% of patients had low serum levels of C3. Serum levels of C3 and non-IgG4 IgG, calculated as the total IgG minus IgG4, showed an inverse correlation in patients with kidney lesions, while serum IgG4 levels were not correlated with serum C3 levels. Corticosteroid was administered in 78.0% of patients, and was effective in all. The serum CRP level is generally low and the serum IgG4 level is elevated in most Japanese IgG4-RD patients, in contrast to western patients. These original findings suggest that these two parameters in IgG4-RD differ in some interesting ways from those hitherto reported in western populations. Additional studies, especially international comparative ones, are needed to elucidate the extent and significance of these differences between populations. Attention will also have to be paid to whether the existence of such differences requires consideration when devising international classification criteria. The online version of this article (doi:10.1186/s13075-017-1467-x) contains supplementary material, which is available to authorized users.
登录
查看更多内容
影响因子：
3.3
作者：
Chang, Sing Yun;Keogh, Karina A.;Yi, Eunhee S.
通讯作者：
Yi, Eunhee S.
影响因子：
4.9
作者：
Mizushima I;Inoue D;Yamamoto M;Yamada K;Saeki T;Ubara Y;Matsui S;Masaki Y;Wada T;Kasashima S;Harada K;Takahashi H;Notohara K;Nakanuma Y;Umehara H;Yamagishi M;Kawano M
通讯作者：
Kawano M
影响因子：
158.5
作者：
Hamano, H;Kawa, S;Kiyosawa, K
通讯作者：
Kiyosawa, K
影响因子：
2.1
作者：
Campochiaro, C.;Ramirez, G. A.;Della-Torre, E.
通讯作者：
Della-Torre, E.
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。
影响因子：
5.5
作者：
Lin, Wei;Lu, Sha;Lipsky, Peter E.
通讯作者：
Lipsky, Peter E.