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目的总结Ig G4相关性疾病在中国患者的临床特点、诊断及治疗,提高对该病的认识和临床诊治。方法对解放军总医院2012年1月至2014年9月期间诊断为Ig G4相关性疾病的患者的临床资料进行回顾性分析。结果 54例患者被诊断为Ig G4相关性疾病,其中男40例(74.1%),女14例;平均年龄为(58.6±1.6)岁(35~82岁);31例(57.4%)出现多器官受累,中位受累器官为2个(2~5个),受累组织包括胰腺(51.9%)、颌面部腺体(48.1%)、淋巴结(20.4%)、胆管(18.5%)等。94%(51/54)的患者伴有血清Ig G4[6.9 g/L(1.4~59.5 g/L)]、Ig G、Ig E等升高为主的高球蛋白血症,97.4%(37/38)患者糖皮质激素治疗后症状有所缓解或恢复正常。结论 Ig G4相关性疾病多见于中老年男性,可累及身体多个部位或组织,以胰腺、颌面部腺体受累最为多见,糖皮质激素治疗有较高疗效。
Objective To summarize the clinical features, diagnosis and treatment of Ig G4 related diseases in Chinese patients and to improve the understanding and clinical diagnosis and treatment of this disease. Methods The clinical data of patients diagnosed as Ig G4-related diseases from January 2012 to September 2014 in People’s Liberation Army General Hospital were retrospectively analyzed. Results Fifty-four patients were diagnosed as Ig G4-related diseases, including 40 males (74.1%) and 14 females with an average age of (58.6 ± 1.6) years (35-82 years) and 31 patients (57.4%) Organ involvement, the median involvement of the organ is 2 (2 ~ 5), involved organizations including the pancreas (51.9%), maxillofacial glands (48.1%), lymph nodes (20.4%), bile duct (18.5%) and so on. Ninety-four percent (51/54) of patients had elevated serum levels of Ig G4 [6.9 g / L (1.4-59.5 g / L)], Ig G and Ig E, and 97.4% (37 / 38) Patients with glucocorticoid symptoms have eased or returned to normal. Conclusion Ig G4-related diseases are more common in middle-aged and elderly men and may involve multiple parts or tissues of the body. The most common pancreatic and maxillofacial gland involvement is glucocorticoid therapy.