IgG4相关性肺疾病二例并文献复习

IgG4-related lung disease： a clinical analysis and literature review

目的总结IgG4相关性肺疾病的临床特点及诊治经验，提高临床对该病的认识，减少误诊误治。方法分析第二军医大学附属长海医院呼吸内科收治的两例IgG4相关性肺疾病的一般情况、临床表现、实验室检查、组织病理学、治疗及预后，并进行相关文献复习。结果两例患者均为男性，血清IgG4水平均显著升高（2．25g／L；10g／L）。1例肺部影像学呈实性结节型改变，并有回盲部受累，糖皮质激素治疗有效。另1例肺部影像学呈支气管血管束型改变，对糖皮质激素治疗依赖。文献复习69例IgG4相关性肺疾病，因呼吸道症状就诊者39例（56．5％），伴有肺外受累者41例（59．4％）。48例检测了血清IgG4水平，均显著升高（307～52500mg／L）。胸部影像学表现实性结节型35例（50．7％）。31例（44．9％）患者接受了糖皮质激素治疗，预后良好。结论IgG4相关性肺疾病是临床少见的免疫性疾病，临床表现缺乏特异性，临床诊断应综合分析其血清免疫学检查、影像学表现及组织病理学结果，糖皮质激素治疗效果良好。

Objective To summarize the clinical features, diagnosis and treatment experience of IgG4-related lung disease for the sake of improving clinical understanding of this disease and reducing the misdiagnosis and mistreatment rates. Methods To analyze the general situation, clinical manifestation, laboratory examination, histopathology, therapy and prognosis of 2 patients with IgG4-related lung disease, who were admitted in the department of respiratory diseases at Changhai Hospital. Publications related to IgG4 lung disease were reviewed. Results Both patients were male with elevated serum IgG4 level（2. 25 g/L and 10 g/L respectively）. In one patient, radiologic finding showed solid nodules in the lung with ileocecal involvement. He responded well to glucocorticoid. The other patient＇s computed tomography of lung demonstrated bronchovascular type. Glucocorticoid therapy was effective to both patients. A total of 69 cases with IgG4-related lung disease were reported worldwide, among whom 39 cases were admitted with chief complaints of respiratory symptoms. However, there were 41 cases suffering extra-pulmonary involvement. Serum IgG4 levels detected in 48 cases were significantly elevated （ 307 - 52 500 mg/L）. The radiographic pattern of solid nodule type was the most frequent, accounting for 50. 7% （35 cases）. A total of 31 （44. 9% ） patients received glucocorticoid therapy with good response and prognosis. Conclusion IgG4- related lung disease is a rare immunological disease lack of specific symptoms. Serum immunological examination, radiographic characteristics and histopathology should be comprehensively considered for diagnosis. Glucocorticoid is so far the most acceptable therapy with good response rate.