Masaki Yasufumi, Kurose Nozomu, Yamamoto Motohisa, Takahashi Hiroki, Saeki Takako, Azumi Atsushi, Nakada Shinji, Matsui Shoko, Origuchi Tomoki, Nishiyama Susumu, Yamada Kazunori, Kawano Mitsuhiro, Hirabayashi Akira, Fujikawa Keita, Sugiura Tomoko, Horikoshi Masanobu, Umeda Naoto, Minato Hiroshi, Nakamura Takuji, Iwao Haruka, Nakajima Akio, Miki Miyuki, Sakai Tomoyuki, Sawaki Toshioki, Kawanami Takafumi, Fujita Yoshimasa, Tanaka Masao, Fukushima Toshihiro, Eguchi Katumi, Sugai Susumu, Umehara Hisanori
Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku-gun, Ishikawa 920-0293, Japan.
Int J Rheumatol. 2012;2012:580814. doi: 10.1155/2012/580814. Epub 2012 May 10.
IgG4-related disease is a new disease classification established in Japan in the 21st century. Patients with IgG4-related disease display hyper-IgG4-gammaglobulinemia, massive infiltration of IgG4+ plasma cells into tissue, and good response to glucocorticoids. Since IgG4 overexpression is also observed in other disorders, it is necessary to diagnose IgG4-related disease carefully and correctly. We therefore sought to determine cutoff values for serum IgG4 and IgG4/IgG and for IgG4+/IgG+ plasma cells in tissue diagnostic of IgG4-related disease. Patients and Methods. We retrospectively analyzed serum IgG4 concentrations and IgG4/IgG ratio and IgG4+/IgG+ plasma cell ratio in tissues of 132 patients with IgG4-related disease and 48 patients with other disorders. Result. Serum IgG4 >135 mg/dl demonstrated a sensitivity of 97.0% and a specificity of 79.6% in diagnosing IgG4-related disease, and serum IgG4/IgG ratios >8% had a sensitivity and specificity of 95.5% and 87.5%, respectively. IgG4+cell/IgG+ cell ratio in tissues >40% had a sensitivity and specificity of 94.4% and 85.7%, respectively. However, the number of IgG4+ cells was reduced in severely fibrotic parts of tissues. Conclusion. Although a recent unanimous consensus of all relevant researchers in Japan recently established the diagnostic criteria for IgG4-related disease, findings such as ours indicate that further discussion is needed.
IgG4相关性疾病是21世纪在日本确立的一种新的疾病分类。IgG4相关性疾病患者表现为高IgG4球蛋白血症、IgG4 +浆细胞大量浸润组织以及对糖皮质激素反应良好。由于在其他疾病中也观察到IgG4过表达，因此有必要仔细、正确地诊断IgG4相关性疾病。因此，我们试图确定用于诊断IgG4相关性疾病的血清IgG4、IgG4/IgG以及组织中IgG4 + /IgG +浆细胞的临界值。患者与方法。我们回顾性分析了132例IgG4相关性疾病患者和48例其他疾病患者的血清IgG4浓度、IgG4/IgG比值以及组织中IgG4 + /IgG +浆细胞比值。结果。血清IgG4>135mg/dl在诊断IgG4相关性疾病时的敏感性为97.0%，特异性为79.6%，血清IgG4/IgG比值>8%时的敏感性和特异性分别为95.5%和87.5%。组织中IgG4 +细胞/Igg +细胞比值>40%时的敏感性和特异性分别为94.4%和85.7%。然而，在组织严重纤维化部位，IgG4 +细胞数量减少。结论。尽管日本所有相关研究人员最近达成的一致共识确立了IgG4相关性疾病的诊断标准，但我们这样的研究结果表明仍需进一步讨论。
