Clinical practice guideline for the diagnosis of granulomatous-lymphocytic interstitial lung disease in patients with common variable immunodeficiency disorders: an ERS Clinical Research Collaboration.

Despite growing recognition of GLILD, there is no accepted diagnostic and management guideline, and current practice varies significantly across centres and countries.

尽管对GLILD的认识日益增加，但尚未有公认的诊断和管理指南，目前不同中心和国家的做法差异很大。

This clinical practice guideline provides evidence- and consensus-based recommendations on the screening and diagnosis of GLILD in patients with CVID.

本临床实践指南提供了基于证据和共识的建议，关于在CVID患者中筛查和诊断GLILD。

Methods

A panel representing multiple interdisciplinary perspectives convened with methodologists to prioritise clinical questions, and review and assess the evidence using Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology.

一个代表多个跨学科视角的小组与方法学家一起召开会议，确定临床问题的优先顺序，并使用Grading of Recommendations Assessment, Development and Evaluation（GRADE）方法审查和评估证据。

Evidence-to-decision frameworks were used to decide on the direction and strength of recommendations.

使用证据到决策框架来决定推荐的方向和强度。

Results

Screening for GLILD is recommended in all adult patients with CVID, preferably using high-resolution computed tomography.

建议在所有成年CVID患者中进行GLILD筛查，首选使用高分辨率计算机断层扫描。

Evaluation should be performed by a multidisciplinary team.

评估应由多学科团队进行。

Routine lung biopsies are not mandatory but necessary for atypical presentations.

常规肺活检不是强制性的，但对于非典型表现是必要的。

As part of the diagnostic process, pulmonary infections should be excluded and lymphocytic alveolitis sought for by means of bronchoalveolar lavage.

作为诊断过程的一部分，应排除肺部感染，并通过支气管肺泡灌洗术寻找淋巴细胞性肺泡炎。

The severity of lung function impairment should be evaluated using pulmonary function tests including gas transfer assessment.

应使用包括气体转移评估在内的肺功能测试来评估肺功能损害的严重程度。

Most of the recommendations are graded as conditional because of low certainty in the evidence regarding health effects.

由于关于健康影响的证据确定性低，大多数建议被定为条件性。

Conclusions

This guideline allows for international homogeneity in the diagnosis of GLILD, thereby paving the way for improved comparability between centres, improving equity in healthcare for those affected by GLILD and facilitating multicentre research collaborations for future studies.

本指南允许在诊断GLILD方面实现国际一致性，从而为不同中心之间的比较铺平道路，改善受GLILD影响者的医疗公平性，并促进未来多中心研究合作。