Background

Common Variable Immunodeficiency (CVID) is characterized by an impaired antibody production and a higher susceptibility to encapsulated bacterial infections. Lung disease is considered to be the most important cause of morbidity and mortality.

Methods

We analyzed clinical, radiological and functional characteristics in 80 patients with CVID assisted in the Unidad Inmunologia e Histocompatibilidad at Durand Hospital from 1982 to 2018.

Results

Of the 80 patients, 55 showed pathologic lung Computed Tomography (CT). Twenty of them (36.4%) showed bronchiectasis; 26 (47.3%) interstitial involvement associated with nodules and adenopathies called GLILD (granulomatous-lymphocytic interstitial lung disease); and nine patients (16.3%) showed other lesions. Nine percent of patients with lung disease showed CT progression; none of them had spirometry worsening. GLILD patients had normal and restrictive patterns in lung function tests, in equal proportions. Two patients – one with GLILD and the other one with bronchiectasis – had an increase in spirometric pattern severity without CT progression. Lung biopsy was performed in 19% of GLILD patients, all of whom had histopathologic diagnosis of Lymphoid Interstitial Pneumonia (LIP).

Conclusions

GLILD is the major cause of lung disease in CVID. Computed tomography is useful for diagnosis but not necessary in follow-up, in which functional tests should have better correlation with clinical evolution, reducing radiation exposure. Biopsy should be indicated when the clinical diagnosis is unclear. Treatment should be considered whenever there is clear evidence of disease progression.

中文翻译：

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肺病患者常见变异免疫缺陷。

背景

常见变异免疫缺陷 (CVID) 的特征是抗体产生受损和对包膜细菌感染的敏感性更高。肺病被认为是发病率和死亡率的最重要原因。

方法

我们分析了 1982 年至 2018 年在杜兰德医院 Unidad Inmunologia e Histocompatibilidad 协助的 80 名 CVID 患者的临床、放射学和功能特征。

结果

在 80 名患者中，55 名显示病理性肺计算机断层扫描 (CT)。其中20人（36.4%）表现出支气管扩张；26 (47.3%) 间质受累与结节和腺病相关，称为 GLILD（肉芽肿性淋巴细胞间质性肺病）；9 名患者（16.3%）表现出其他病变。9% 的肺部疾病患者出现 CT 进展；他们都没有肺功能恶化。GLILD 患者在肺功能测试中具有正常和限制性模式，比例相等。两名患者 - 一名患有 GLILD，另一名患有支气管扩张 - 肺活量模式严重程度增加，而 CT 没有进展。19% 的 GLILD 患者进行了肺活检，所有这些患者的组织病理学诊断为淋巴样间质性肺炎 (LIP)。

结论

GLILD 是 CVID 中肺部疾病的主要原因。计算机断层扫描对诊断有用，但在随访中不是必需的，其中功能测试应与临床演变有更好的相关性，减少辐射暴露。临床诊断不明确时应进行活检。只要有明确的疾病进展证据，就应考虑治疗。