【摘要】 目的探讨IgG4相关性肺疾病的临床表现、影像学表现、病理表现、诊疗及预后。方法回顾性分析福建省立医院呼吸与危重症医学科2018年11月收治的1例IgG4相关性肺疾病患者的临床资料并进行相关文献复习,以"IgG4相关性肺疾病"为检索词检索万方数据库和中国期刊全文数据库,以"IgG4related lung disease"为检索词检索PubMed、Embase、Ovid及Cochrane数据库,检索时间为2007年1月至2019年10月。结果患者68岁男性,以"咳嗽、咳痰伴发热20天"为主诉于2018年11月27日入院,表现为咳嗽、咳黄脓痰、持续性低热,入院后查血清IgG4 13.60g/L,胸部CT示左肺多发病变,完善肺活检病理示多量浆细胞浸润,免疫组化IgG4>40%(每高倍镜30个),确诊为IgG4相关性肺疾病,以甲强龙治疗后随访半年,病灶较前明显吸收,目前仍在治疗中。共检索到相关文献77篇,统计后显示男性发病率较女性高,平均年龄(57.2±15.1)岁,临床表现无特异性。肺部影像学多为肺部结节、实变型和磨玻璃影型。74.68%的患者血清IgG升高,86.27%患者血清IgG4升高,所有患者病理结果显示IgG4均阳性。治疗上单用激素复发率为5.81%,激素联合免疫抑制剂治疗的复发率及死亡率较低。结论 IgG4相关性肺疾病临床表现及胸部影像学无特异性,血清IgG及IgG4升高对诊断有提示意义,最终需病理确诊;糖皮质激素是首选治疗药物,治疗效果不佳或复发时可联合免疫抑制剂,可减少复发率和死亡率。更多还原

【Abstract】 Objective To analyze the clinical features,imaging findings,pathological manifestation,diagnosis and prognosis of IgG4-related lung diseases.Methods The clinical features,imaging findings,pathological manifestation,diagnosis and prognosis of a case with IgG4-related pulmonary disease were reported and related literatures were reviewed.The literature review was carried out respectively in PubMed,Embase,Ovid and Cochrane from January 2007 to October 2019 with"IgG4 related lung disease"being the search terms.Results A 68 year-old-male patient was admitted to the hospital on November 27,2018,because of productive cough and fever for 20 days.The patient showed cough,purulent sputum and persistent fever.His serum IgG4 was 13.60 g/L,computed tomography(CT)of the chest revealed multiple lesions in left lobes,lung biopsy was performed in the left upper lobe.Histological examination showed multiple plasma cell infiltration throughout the alveolar wall.IgG4-immunopositive cells were observed and the average number of these cells was 30 per high power field(HPF),which was more than 40%.We considered IgG4-related lung disease to be the diagnosis and treated the patient with methylprednisolone.The patients condition improved and he is under regular follow-up.A total of 77 literatures were retrieved from PubMed,Embase,Ovid and Cochrane.The incidence of male was higher than that of female.Fever,cough,dyspnea and thoracalgia were the most common symptoms.CT findings could be categorized into two major subtypes:solid nodular and round-shaped ground glass opacity(GGO).74.68% patients showed an elevated serum IgG,and 86.27% patients showed an elevated serum IgG4.And pathological manifestation showed IgG4-positive cells in all patients.The rate of recurrence in steroid therapy was 5.81%,and steroid therapy combined immunotherapy could reduce both recurrence and mortality rate.Conclusion The diagnosis of IgG4-related lung disease requires a combination of clinical history,physical examination,imaging findings and pathological manifestation.It has been known that steroid therapy was the first-line therapy and could reduce recurrence and mortality rate when combined with immunotherapy.更多还原