IgG4相关性肺疾病八例临床特征分析

IgG4-related lung disease：analysis of 8 cases and literature review

目的 提高对IgG4相关性肺疾病（IgG4-RLD）的认识及诊治水平.方法 回顾性分析2005年12月至2016年3月于解放军总医院住院并经病理确诊为IgG4-RLD的8例患者的临床特点、血清IgG4水平、病理特征、胸部CT、治疗及预后并进行文献复习（2006年1月至2016年10月Pubmed数据库、中国知网及万方数据库）.结果 8例IgG4-RLD患者中,女性4例,男性4例,年龄37~74岁,平均（59±4）岁.呼吸道症状：活动后气短2例,咳嗽2例,咳痰2例;肺外症状：腹痛5例,颜面水肿2例,发热1例.7例患者合并肺外受累.所有患者血清IgG4水平均升高,平均浓度为（17±6）g/L.胸部CT显示肺实性结节型6例,肺泡间质型5例,支气管血管束型3例,磨玻璃影型2例.2例行PET/CT检查提示为多脏器受累,病变部位放射性摄取升高,最大标准摄取值（SUVmax）为2.9~4.2.病理表现：7例为大量淋巴细胞、浆细胞浸润,5例纤维组织增生,2例闭塞性血管炎.8例中免疫组织化学IgG4/IgG〉40%者3例,IgG4阳性细胞绝对值均为10~50个/HP.病理确诊前误诊率100%.3例接受糖皮质激素（激素）联合免疫抑制剂治疗,2例病变部位手术切除联合激素治疗,2例单纯激素治疗,1例单纯手术治疗.随访4~132个月,7例病情缓解,1例病情进展,无死亡病例.文献复习195例IgG4-RLD,男女比例为2.2：1,平均年龄（57±15）岁.以呼吸道症状就诊者111例,肺外受累者144例.179例患者检测血清IgG4浓度,平均浓度为5.408 g/L.胸部影像学表现以结节型居多（36.9%）.178例（91.3%）患者经糖皮质激素治疗后病情缓解.结论 IgG4-RLD的主要临床表现为活动后气短、咳嗽及咳痰;病变常累及多器官;确诊主要依据病变部位病理组织活检、免疫组织化学检测及血清IgG4水平;该病误诊率极高,糖皮质激素治疗有效.

Objective To improve the understanding and treatment of IgG 4-related lung disease （IgG4-RLD）.Methods The clinical characteristics, serum IgG4 levels, pathological features, chest CT, therapy and prognosis of 8 patients with IgG4-RLD were retrospectively analyzed . These patients were admitted to the People ＆#39; s Liberation Army General Hospital and the pathological diagnosis was made between December 2005 and March 2016.Relevant literatures were reviewed .Results The 8 patients with IgG4-RLD included 4 men and 4 women, with an average age of （ 59 ±4 ） years （ range, 37 -74 ） .The respiratory symptoms included shortness of breath , cough, and expectoration.Extra-pulmonary symptoms included abdominal pain , facial edema , and fever.Extrapulmonary organs were involved in 7 cases.Serum IgG4 levels were elevated in 8 cases, with an average concentration of （17 ±6）g/L.Chest CT showed solid lung nodules in 6, alveolar-interstitial infiltration in 5, bronchovascular lesions in 3 and ground glass shadows in 2 cases.PET/CT was performed in 2 cases and it showed multiple organ involvement with higher radioactivity uptake （ SUVmax2.9-4.2 ） .The pathological examination found lymphocyte and plasma cell infiltration in 7, fibrous tissue hyperplasia in 5, and occlusive vasculitis in 2 cases. On immunohistochemical staining , the ratio of IgG4-positive plasma cells to IgG-positive plasma cells was higher than 40%in 3 cases. The number of IgG4-positive plasma cells was 10 -50/HP in 8 cases. The misdiagnosis rate was 100%before the final diagnosis was made .Three cases received glucocorticoids with immunosuppressant therapy , 2 received surgery combined with glucocorticoid therapy , 2 received glucocorticoid therapy alone, and 1 only received surgery.The follow-up time was 4 -132 months, with remission in 7 cases, and disease progression in 1 case,but no death.A total of 195 cases of IgG4-RLD were reviewed from the literature , among whom 111 cases were admitted with respiratory symptoms , 144 with extra-pulmonary involvement.Serum IgG4 levels were detected in 179 cases, with an average concentration of 5.408 g/L.The nodular type was predominant , accounting for 36.9%.Of these cases , 178 received glucocorticoid treatment with disease remission .Conclusions The major clinical manifestations of IgG 4-RLD were shortness of breath , cough and expectoration . Multiple organ lesions were common . The misdiagnosis rate was extremely high .The diagnosis could be made based on pathological features and IgG 4 serum levels .Glucocorticoid treatment was effective .