Peng Tao, Hu Zhao, Xie Tingting, Jiang Baodong, Zhen Junhui, Yang Xiangdong
Department of Nephrology, Shandong University Qilu Hospital, Jinan, 250012 China.
Springerplus. 2016 Jun 21;5(1):804. doi: 10.1186/s40064-016-2537-2. eCollection 2016.
IgG4-related disease was first reported in 2001 and was officially named in 2010. It is now considered as a systemic disease that might affect any organ system. The characteristic pathological changes of IgG4-related disease are extensive infiltration of IgG4-positive plasma cells. IgG4-related disease is a kind of benign lesions, but there has not been well-defined standard treatment so far. Patients usually respond well to corticosteroids. The prognosis of IgG4-related disease is perhaps good as long as early detection and treatment.
We report one case of IgG4-related disease with a 16-years anamnesis with multi-pseudotumor masses. He was diagnosed with chronic kidney disease with wide interstitial renal fibrosis. And he received glucocorticoids therapy. After 2 month therapy, the serum creatinine, erythrocyte sedimentation rate, and serum IgG4 decreased significantly. The discussion includes presentation, clinical course, diagnosis, and prognosis of IgG4-related disease. The case and discussion highlight the importance of diagnosis and the good prognosis of IgG4-related diseases.
Our case highlights the importance of diagnosis and the good prognosis of IgG4-related diseases. IgG4-related disease is a systemic fibro-inflammatory immune-mediated disorder and now recognized in almost every major organs. Characteristics of the disease is multiple lymph nodes and the response to glucocorticoids therapy is well. In such case, he had a history of 16 years with multi-pseudotumor masses and misdiagnosed for 16 years, if the doctors were not awareness of higher serum immunoglobulin G4 (IgG4) than normal, the correct diagnosis may be missed or delayed. Consequently, appropriate treatment for IgG4-related disease would also be delayed or not provided and likely result in increased morbidity and mortality.
IgG4-related disease is a systemic fibro-inflammatory immune-mediated disorder and progresses slowly. In the present patient the course of IgG4-related disease appears to be benign. The prognosis of IgG4-related disease depend on early diagnosis and treatment.
IgG4相关性疾病于2001年首次报道，并于2010年正式命名。它现在被认为是一种可能影响任何器官系统的全身性疾病。IgG4相关性疾病的特征性病理变化是IgG4阳性浆细胞的广泛浸润。IgG4相关性疾病是一种良性病变，但迄今为止尚未有明确的标准治疗方法。患者通常对皮质类固醇反应良好。只要早期发现并治疗，IgG4相关性疾病的预后可能良好。
我们报告一例有16年病史的IgG4相关性疾病患者，伴有多个假瘤肿块。他被诊断为慢性肾脏病，伴有广泛的肾间质纤维化。他接受了糖皮质激素治疗。治疗2个月后，血清肌酐、红细胞沉降率和血清IgG4显著下降。讨论内容包括IgG4相关性疾病的表现、临床过程、诊断和预后。该病例及讨论强调了IgG4相关性疾病诊断的重要性以及良好的预后。
我们的病例强调了IgG4相关性疾病诊断的重要性以及良好的预后。IgG4相关性疾病是一种全身性纤维炎性免疫介导的疾病，现在几乎在每个主要器官中都有发现。该疾病的特点是多个淋巴结肿大，对糖皮质激素治疗反应良好。在这种情况下，他有16年多假瘤肿块病史且误诊了16年，如果医生没有意识到血清免疫球蛋白G4（IgG4）高于正常水平，可能会漏诊或延误正确诊断。因此，IgG4相关性疾病的适当治疗也会被延误或未给予，可能导致发病率和死亡率增加。
IgG4相关性疾病是一种全身性纤维炎性免疫介导的疾病，进展缓慢。在本患者中，IgG4相关性疾病的病程似乎是良性的。IgG4相关性疾病的预后取决于早期诊断和治疗。
