Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-Ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Republic of Korea.
Respir Res. 2020 Oct 19;21(1):273. doi: 10.1186/s12931-020-01542-6.
Immunoglobulin G4-related lung disease (IgG4-RLD) is the pulmonary manifestation of a systemic fibroinflammatory disease characterized by lymphoplasmacytic infiltration with an abundance of IgG4-positive plasma cells. Long-term clinical course and outcomes of IgG4-RLD remain unclear. We aimed to identify clinical characteristics, treatment outcomes, and longitudinal pulmonary function changes in patients with IgG4-RLD according to the radiologic classification.
Chest computed tomography findings of 37 subjects were classified into five subtypes: solid nodular, bronchovascular, alveolar interstitial, round ground glass opacity, and alveolar consolidative. Radiologic treatment outcomes and longitudinal pulmonary function changes were compared among the different radiologic subtypes.
The mean age of the subjects was 55.6 years, and 78.4% were male. Among the five radiologic subtypes, alveolar consolidative and solid nodular type were most common, accounting for approximately 29.7% each of the total cases. Prednisone with or without azathioprine was administered to 31 patients (median treatment duration 14 months). In the treated patients, serial images showed complete response or partial response in 77.4%. However, relapse was documented in 25.0% of those who showed complete or partial response. In patients whose longitudinal lung function data were available (n = 20), the lung function was found to be stable during follow-up. Alveolar consolidative type showed the highest complete response rate, whereas alveolar interstitial type showed the lowest response rate, either complete or partial.
Most patients showed a favorable outcome with regards to radiologic improvement and maintenance of pulmonary function; however, the response differed according to the radiologic subtype.
免疫球蛋白 G4 相关肺病（IgG4-RLD）是一种系统性纤维炎症性疾病的肺部表现，其特征为淋巴浆细胞浸润伴大量 IgG4 阳性浆细胞。IgG4-RLD 的长期临床病程和结局尚不清楚。我们旨在根据影像学分类确定 IgG4-RLD 患者的临床特征、治疗结局和纵向肺功能变化。
对 37 例患者的胸部计算机断层扫描（CT）结果进行分类，分为 5 种亚型：实性结节、支气管血管、肺泡间质、圆形磨玻璃影和肺泡实变。比较不同影像学亚型之间的放射学治疗结局和纵向肺功能变化。
患者的平均年龄为 55.6 岁，78.4%为男性。在五种影像学亚型中，肺泡实变和实性结节型最常见，占总病例数的 29.7%左右。31 例患者（中位治疗时间 14 个月）接受了泼尼松联合或不联合硫唑嘌呤治疗。在接受治疗的患者中，77.4%的患者影像学显示完全缓解或部分缓解。然而，在那些影像学显示完全或部分缓解的患者中，有 25.0%出现了复发。在有纵向肺功能数据的患者（n=20）中，在随访期间发现肺功能稳定。肺泡实变型的完全缓解率最高，而肺泡间质型的缓解率最低，无论是完全缓解还是部分缓解。
大多数患者在影像学改善和肺功能维持方面表现出良好的结局；然而，反应因影像学亚型而异。
