Clinical Practice Guideline for the diagnosis of Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) in patients with Common Variable Immunodeficiency Disorders (CVID) – 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. OBJECTIVE: 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 prioritize clinical questions, review and assess the evidence using GRADE (Grading of Recommendations Assessment, Development and Evaluation) 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 CVID patients, 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 after 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 centers, improving equity in health care for those affected by GLILD and facilitating multicenter research collaborations for future studies.

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Publication Details

Journal
European Respiratory Journal
Published
2026-06-25
DOI
https://doi.org/10.1183/13993003.02224-2025
Primary Topic
Immunodeficiency and Autoimmune Disorders
Type
article
Field-Weighted Citation Impact
0.00

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article

Clinical Practice Guideline for the diagnosis of Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) in patients with Common Variable Immunodeficiency Disorders (CVID) – an ERS Clinical Research Collaboration

Joseph Jacob, Joris M. van Montfrans, Klaus Warnatz, John Robert Hurst et al.
European Respiratory Journal
Immunodeficiency and Autoimmune Disorders
article

Clinical Practice Guideline for the diagnosis of Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) in patients with Common Variable Immunodeficiency Disorders (CVID) – an ERS Clinical Research Collaboration

Joseph Jacob, Joris M. van Montfrans, Klaus Warnatz, John Robert Hurst, Jesper Rømhild Davidsen, Francesco Cinetto, Børre Fevang, Cinzia Milito, María M. Trujillo‐Martín, Annick A. J. M. van de Ven, Mariana Calderón-Jaramillo, Leif G. Hanitsch, Carlyne Cool, Maria X. Rojas-Reyes, Heba M. Bintalib, Paul Maglione, John Routes, Friedolin Strauss, Ximena Lopez-Mujica, Filomeen Haerynck, Marion Malphettes
article en

Abstract

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. OBJECTIVE: 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 prioritize clinical questions, review and assess the evidence using GRADE (Grading of Recommendations Assessment, Development and Evaluation) 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 CVID patients, 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 after 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 centers, improving equity in health care for those affected by GLILD and facilitating multicenter research collaborations for future studies.

European Respiratory Journal
Oslo University Hospital (NO), Boston Medical Center (US), University Medical Center Groningen (NL), University of Freiburg (DE), University of Southern Denmark (DK), University of Oslo (NO), King Saud bin Abdulaziz University for Health Sciences (SA), Hawassa University (ET), Utrecht University (NL), Medical College of Wisconsin (US), Ghent University Hospital (BE), University Medical Center Freiburg (DE), Odense University Hospital (DK), The Medical Center of Aurora (US), University Medical Center Utrecht (NL), Ghent University (BE), Humboldt-Universität zu Berlin (DE), King Abdullah International Medical Research Center (SA), Assistance Publique – Hôpitaux de Paris (FR), University Hospital of Zurich (CH), Red de Investigación en Actividades Preventivas y Promoción de la Salud (ES), Bundesverband Glaukom-Selbsthilfe (DE), Servicio Canario de la Salud (ES), Centre for Demographic Studies (ES), Health Research Institute of the Balearic Islands (ES), Systems, Applications & Products in Data Processing (United Kingdom) (GB), Berlin Institute of Health at Charité - Universitätsmedizin Berlin (DE), Hôpital Saint-Louis (FR), Ca' Foncello Hospital (IT), University College London (GB), National Guard Health Affairs (SA), University of Colorado Anschutz Medical Campus (US), Freie Universität Berlin (DE), Charité - Universitätsmedizin Berlin (DE), Sapienza University of Rome (IT)
Wellcome Trust
Partnerships for the goals
Openalex Percentile: Top 11%
Immunodeficiency and Autoimmune Disorders
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