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摘要: IgG4相关性疾病(IgG4-related disease, IgG4-RD)是累及多器官或组织的慢性进行性自身免疫性疾病, 其病理为受累脏器被淋巴细胞和IgG4阳性的浆细胞浸润, 肺部表现多样。本文报道了一例以咳嗽、气短起病的IgG4-RD, 有肺、纵膈淋巴结、左颌下淋巴结、腹膜后、脾门、胃粘膜、皮肤受累, 其肺野内病变表现为弥漫微结节, 激素治疗后完全消失。Abstract: IgG4-related disease(IgG4-RD) is a chronic autoimmune disease implicating multiple organs and tissues. Its pathological characteristics are infiltration of lymphocytes and IgG4-positive plasma cells in the implicated organs. Pulmonary involvement reportedly varies. Here we reported a case of IgG4-RD with the onset of cough and dyspnea, presenting with multiple lesions. The pulmonary involvement was manifested as diffuse nodules in lungs that dissolved after glucocorticoid therapy.
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Key words:
- IgG4-related disease /
- pulmonary nodule /
- glucocorticoid
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[1] Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease(IgG4-RD)[J]. Mod Rheumatol, 2012, 22:21-30. doi: 10.3109/s10165-011-0571-z [2] Geyer JT, Niesvizky R, Jayabalan DS, et al.IgG4 plasma cell myeloma:new insights into the pathogenesis of IgG4-related disease[J].Mod Pathol, 2014, 27:375-381. doi: 10.1038/modpathol.2013.159 [3] Inoue D, Yoshida K, Yoneda N, et al. IgG4-related disease:dataset of 235 consecutive patients[J].Medicine(Baltimore), 2015, 94:e680. http://cn.bing.com/academic/profile?id=8a4b6cb03b1b854b23f3d478307cca48&encoded=0&v=paper_preview&mkt=zh-cn [4] Inoue D, Zen Y, Abo H, et al. Immunoglobulin G4-related lung disease:CT findings with pathologic correlations[J]. Radiology, 2009, 251:260-70. doi: 10.1148/radiol.2511080965 [5] Matsui S, Hebisawa A, Sakai F, et al. Immunoglobulin G4-related lung disease:Clinicoradiological and pathological features[J]. Respirology, 2013, 18:480-487. doi: 10.1111/resp.12016 [6] Suzuki H, Watanabe M, Ara T, et al. Immunoglobulin G4-related lung disease accompanied by organizing pneumonia[J].Intern Med, 2013, 52:2105-2111. doi: 10.2169/internalmedicine.52.0112 [7] Duvic C, Desrame J, Lévêque C, et al.Retroperitoneal fibrosis, sclerosing pancreatitis and bronchiolitis obliterans with organizing pneumonia[J]. Nephrol Dial Transplant, 2004, 19:2397-2399. doi: 10.1093/ndt/gfh050 [8] Onishi Y, Kawamura T, Kagami R, et al.IgG4-related lung disease with organizing pneumonia effectively treated with azathioprine[J].Intern Med, 2014, 53:2701-2704. doi: 10.2169/internalmedicine.53.2564 -
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