摘要
目的探讨Ig G4相关性肺疾病的临床特点。方法收集解放军总医院2000年1月至2014年1月诊断的2例Ig G4相关性肺疾病患者的临床表现、实验室检查、影像、诊断及治疗资料,并结合文献进行回顾性分析。结果 Ig G4相关性肺疾病的血清总Ig G可正常,Ig G1、Ig G2、Ig G3和Ig G4均可升高,部分自身抗体阳性。累及泪腺、涎腺时容易误诊为干燥综合征伴间质性肺炎,胸部CT偶有多发小囊泡。肺组织免疫化学染色结果示Ig G4/Ig G升高,Ig G4阳性淋巴浆细胞>10个/HP。综合临床表现、实验室检查、影像及病理有助于Ig G4相关性肺疾病的诊断。糖皮质激素治疗后临床症状缓解,血清Ig G4明显下降。结论 Ig G4相关性肺疾病临床较少见,可单独累及肺,也可多器官累及,肺组织免疫化学染色Ig G4/Ig G升高为诊断的金标准。糖皮质激素治疗效果良好。
Objective To summarize the clinical characteristic of Ig G4 related lung disease.Methods The clinical manifestation,laboratory examination,imaging,diagnosis and treatment data of2 patients with Ig G4 related lung disease admitted in the PLA General Hospital from January 2000 to January2014 were collected and analyzed retrospectively. Related literatures were also reviewed. Results The serum Ig G level of Ig G4 related lung disease might be normal and the levels of Ig G1,2,3 and 4 might increase.Some autoantibodies were positive. Ig G4 related lung disease could be easily misdiagnosed as Sjogren’ s syndrome associated with interstitial pneumonia when the lacrimal gland and salivary gland were involved,and the chest CT occasionally showed multiple vesicles. The immunochemical staining of lung tissues revealed the increase of Ig G4 / Ig G and the number of Ig G4 positive lymphatic plasma cells 〉 10 / HP. The combination of clinical manifestation,laboratory examination,imaging and pathology results was more conducive for the diagnosis of Ig G4 related lung disease. After the treatment of glucocorticoid,the clinical symptoms were relieved and the serum Ig G4 level obviously decreased. Conclusions Ig G4 related lung disease is rare in clinic,which can involve the lung alone or multiple organs. The increase of Ig G4 / Ig G indicated by the immunochemical staining of lung tissues is the gold standard for diagnosis. Glucocorticoid has good treatment effect in Ig G4 related lung disease.
出处
《中国呼吸与危重监护杂志》
CAS
2014年第6期579-583,共5页
Chinese Journal of Respiratory and Critical Care Medicine
基金
军队中医药科研专项课题(编号:413EG63E)
