Department of Pneumology, University Medical Clinic Freiburg, Freiburg, Germany.
Curr Opin Pulm Med. 2013 Sep;19(5):503-9. doi: 10.1097/MCP.0b013e3283642c47.
Common variable immunodeficiency (CVID) is the most common primary immunodeficiency characterized by a deficiency of immunoglobulins. Approximately 30% of the patients develop autoimmune and granulomatous disease. Similar to sarcoidosis, granulomatous disease in CVID can potentially affect all organs, but the lung is the most common. Interstitial lung disease (ILD) manifests in 5-15% of CVID patients, and is present already at the initial diagnosis in the majority of patients. The number of published studies addressing ILD in CVID is limited. However, recently, several studies added substantial knowledge to the field and are discussed within this review in the context of the literature.
Histologically, ILD in CVID presents within the known patterns of sarcoid-like granuloma, organizing pneumonia, lymphocytic interstitial pneumonitis and nonspecific interstitial pneumonia. Often, these patterns are concomitantly found in the same patients. Three new articles were published which analyzed high-resolution computed tomography findings and response to treatment.
In a considerable number of patients, ILD is stable over years and patients may not need any immunosuppressive treatment. Prednisone treatment is often used as the first-line treatment and studies suggest response to treatment in 50-66% of cases. In progressive disease with lung function impairment, combined immunosuppressive treatment is recommended.
普通变异性免疫缺陷症（CVID）是最常见的原发性免疫缺陷症，其特征是免疫球蛋白缺乏。大约 30%的患者会发展出自身免疫和肉芽肿性疾病。与结节病类似，CVID 中的肉芽肿性疾病可能会影响所有器官，但肺部最常见。间质性肺病（ILD）在 5-15%的 CVID 患者中表现出来，并且在大多数患者的初始诊断中已经存在。针对 CVID 中 ILD 的已发表研究数量有限。然而，最近，有几项研究在该领域增加了大量知识，并在本文中结合文献进行了讨论。
组织学上，CVID 中的 ILD 表现为已知的类肉瘤样肉芽肿、机化性肺炎、淋巴细胞性间质性肺炎和非特异性间质性肺炎模式。通常，这些模式同时存在于同一患者中。有三篇新文章分析了高分辨率计算机断层扫描结果和治疗反应。
在相当多的患者中，ILD 多年来保持稳定，患者可能不需要任何免疫抑制治疗。泼尼松治疗通常用作一线治疗，研究表明 50-66%的病例有治疗反应。在伴有肺功能损害的进行性疾病中，建议联合免疫抑制治疗。
