Intrathoracic Involvements of Immunoglobulin G4-Related Sclerosing Disease.
Intrathoracic Involvements of Immunoglobulin G4-Related Sclerosing Disease.
复制标题
免疫球蛋白 G4 相关硬化性疾病的胸腔内受累。
DOI：
10.1097/md.0000000000002150
复制
发表时间：
2015-12
期刊：
影响因子：
1.6
通讯作者：
Zhang F
中科院分区：
文献类型：
--
作者：
Fei Y;Shi J;Lin W;Chen Y;Feng R;Wu Q;Gao X;Xu W;Zhang W;Zhang X;Zhao Y;Zeng X;Zhang F
目的探讨IgG 4相关疾病（IgG 4-RD）胸内受累的临床和影像学特点。对2011年1月至2015年3月在北京协和医院入组的IgG 4-RD患者进行前瞻性队列研究，总结IgG 4-RD胸内受累患者的临床和影像学特征。248例IgG 4-RD患者中，87例为胸内病变，其中男性58例，女性29例，平均年龄54.19 ± 13.80岁。 肺门和纵隔淋巴结肿大是IgG 4相关性胸腔疾病最常见的表现，占52.9%（46/87）。肺部病变的其他影像学表现包括：实性结节（25.3%）、圆形磨玻璃影（9.2%）、肺泡间质型（20.7%）、支气管血管型（23.0%）、胸腔积液（4.6%）和胸膜结节或增厚（16.1%）。只有27名患者出现呼吸道症状，包括咳嗽、呼吸困难、胸痛和哮喘。与无胸内疾病的患者相比，IgG 4相关性胸内疾病患者的IgG 4和C反应蛋白水平较高，过敏、发热和多器官受累的发生率较高。大多数肺间质性疾病、纵膈肿块和支气管增厚对皮质类固醇和免疫抑制剂治疗敏感，而36.3%（8/22）的孤立性结节性病变对治疗无反应。8例患者未接受治疗，5例保持稳定，2例患者自发改善，1例患者失访。IgG 4-RD患者的胸腔内病变并不罕见，包括支气管增厚、结节、磨玻璃样阴影、胸膜增厚/积液、淋巴结病等。大多数肺间质病变、纵隔肿块和支气管增厚患者均观察到皮质类固醇和免疫抑制剂治疗的疗效。
To investigate clinical and radiological features of IgG4-related disease (IgG4-RD) patients with intrathoracic involvement. A prospective cohort study was performed and IgG4-RD patients were enrolled from January 2011 to March 2015 in Peking Union Medical College Hospital, in which the clinical and radiological characteristics of IgG4-RD patients with intrathoracic involvement were summarized. Out of total 248 cases with IgG4-RD, 87 cases had intrathoracic lesions, including 58 male cases and 29 female cases, with average age of 54.19 ± 13.80 years. Hilar and mediastinal lymphadenopathy were the most common manifestations of IgG4-related intrathoracic disease, accounting for 52.9% (46/87). Other imaging findings of pulmonary disease included: solid nodular (25.3%), round-shaped ground-glass opacities (9.2%), alveolar-interstitial type (20.7%), bronchovascular type (23.0%), pleural effusion (4.6%), and pleural nodules or thickening (16.1%). Only 27 patients presented with respiratory symptoms, including cough, breathless, chest pain, and asthma. Compared with patients without intrathoracic disease, IgG4-related intrathoracic disease had higher IgG4 and C-reactive protein level, and higher incidence of allergy, fever, and multi-organ involvement. Most of lung interstitial disease, mediastinal mass, and bronchial thickening were sensitive to corticosteroid and immunosuppressant therapy, while 36.3% (8/22) of solitary nodular lesions were unresponsive to treatment. Eight patients were on no treatment, with 5 cases remained stable, 2 patients improved spontaneously, and 1 patient was lost follow-up. Intrathoracic lesions are not rare in patients with IgG4-RD, involving bronchial thickening, nodules, ground glass opacity, pleural thickening/effusion, lymphadenopathy, etc. Efficacy of corticosteroid and immunosuppressant therapy were noted in most of patients with lung interstitial disease, mediastinal mass, and bronchial thickening.