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

BACKGROUND

Granulomatous-lymphocytic interstitial lung disease (GLILD) is a lung disease first described in people affected by common variable immunodeficiency disorders (CVID). Despite growing recognition of GLILD, there is no accepted diagnostic and management guideline, and current practice varies significantly across centres and countries. This clinical practice guideline provides evidence- and consensus-based recommendations on the screening and diagnosis of GLILD in patients with CVID.

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

RESULTS

Screening for GLILD is recommended in all adult patients with CVID, preferably using high-resolution computed tomography. Evaluation should be performed by a multidisciplinary team. Routine lung biopsies are not mandatory but necessary for atypical presentations. As part of the diagnostic process, pulmonary infections should be excluded and lymphocytic alveolitis sought for by means of bronchoalveolar lavage. The severity of lung function impairment should be evaluated using pulmonary function tests including gas transfer assessment. Most of the recommendations are graded as conditional because of low certainty in the evidence regarding health effects.

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.