Cao Lu, Chen Yi-Bing, Zhao Da-Hui, Shi Wen-Fang, Meng Song, Xie Li-Xin
Department of Pulmonary & Critical Care Medicine, Chinese People's Liberation Army General Hospital, Beijing 100853, China.
J Thorac Dis. 2017 Mar;9(3):547-554. doi: 10.21037/jtd.2017.02.73.
IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory disorder that can affect most organs. To date, there have been no detailed assessments of pulmonary function in patients with IgG4-RD. In this study, we investigated pulmonary function in IgG4-RD patients and evaluated the value of pulmonary function tests (PFTs) in diagnosing IgG4-related respiratory disease (IgG4-RRD).
This was a retrospective study of 17 patients with IgG4-RD. The patients were divided into two groups: IgG4-RRD group and IgG4-related disease extrapulmonary involvement (IgG4-RDEI) group. The PFT results were compared between the two groups.
All patients in the IgG4-RRD group had pulmonary dysfunction. Five of 8 (62.5%) patients in the IgG4-RDEI group had pulmonary dysfunction, despite having normal thoracic computed tomography scans and no respiratory symptoms. Patients in both groups showed restrictive ventilatory dysfunction and abnormal diffusing capacity, and two patients in the IgG4-RRD group had obstructive ventilatory dysfunction. The incidence of diffusing capacity of the lung for carbon monoxide per liter of alveolar volume (DCO/V) decrease were significantly higher in the IgG4-RRD group than in the IgG4-RDEI group (P=0.029). DCO/V were significantly higher in the IgG4-RDEI than in the IgG4-RRD group (P=0.044), but otherwise, there were no significant differences. We report the first finding of a negative correlation between pulmonary diffusing capacity and total serum concentrations of IgG and IgG subclasses (IgG4, IgG3 and IgG2).
DCO/V plays an important role for detecting lung involvement in IgG4-RD patients. The patient with high serum IgG may be more prone to respiratory involvement.
IgG4相关疾病（IgG4-RD）是一种可影响大多数器官的免疫介导的纤维炎症性疾病。迄今为止，尚未对IgG4-RD患者的肺功能进行详细评估。在本研究中，我们调查了IgG4-RD患者的肺功能，并评估了肺功能测试（PFT）在诊断IgG4相关呼吸道疾病（IgG4-RRD）中的价值。
这是一项对17例IgG4-RD患者的回顾性研究。患者分为两组：IgG4-RRD组和IgG4相关疾病肺外受累（IgG4-RDEI）组。比较两组的PFT结果。
IgG4-RRD组所有患者均存在肺功能障碍。IgG4-RDEI组8例患者中有5例（62.5%）存在肺功能障碍，尽管其胸部计算机断层扫描正常且无呼吸道症状。两组患者均表现为限制性通气功能障碍和弥散功能异常，IgG4-RRD组有2例患者存在阻塞性通气功能障碍。IgG4-RRD组每升肺泡容积一氧化碳弥散量（DCO/V）降低的发生率显著高于IgG4-RDEI组（P=0.029）。IgG4-RDEI组的DCO/V显著高于IgG4-RRD组（P=0.044），但除此之外，两组之间无显著差异。我们首次发现肺弥散功能与血清总IgG及IgG亚类（IgG4、IgG3和IgG2）浓度之间存在负相关。
DCO/V在检测IgG4-RD患者的肺部受累中起重要作用。血清IgG水平高的患者可能更容易发生呼吸道受累。
