Clinical practice guideline for the diagnosis of granulomatous-lymphocytic interstitial lung disease in patients with common variable immunodeficiency disorders: an ERS Clinical Research Collaboration
Year: 2026
Type: article
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. 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.
Source: European Respiratory Journal
Authors: Heba M. Bintalib, Annick A.J.M. van de Ven, Maria X. Rojas-Reyes, Jesper R. Davidsen, Leif G. Hanitsch, Francesco Cinetto, Marion Malphettes, Joris M. Van Montfrans, Joseph Jacob, Cinzia Milito, Filomeen Haerynck, Friedolin Strauss, Carlyne Cool, Mariana Calderon-Jaramillo, Ximena Lopez-Mujica, Paul Maglione, Maria M. Trujillo-Martin, Borre Fevang, John Routes, John R. Hurst, Klaus Warnatz
Institutions: King Saud bin Abdulaziz University for Health Sciences, King Abdullah International Medical Research Center, National Guard Health Affairs, University Medical Center Groningen, Servicio Canario de la Salud, University of Southern Denmark, Odense University Hospital, Humboldt-Universität zu Berlin, Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Charité - Universitätsmedizin Berlin, University of Padua, Ca' Foncello Hospital, Assistance Publique – Hôpitaux de Paris, Hôpital Saint-Louis, Utrecht University, University Medical Center Utrecht, University College London, Sapienza University of Rome, Ghent University Hospital, Ghent University, University of Colorado Anschutz, Universitat Autònoma de Barcelona, Centre for Demographic Studies, Boston University, Boston Medical Center, Oslo University Hospital, University of Oslo, Medical College of Wisconsin, University of Freiburg, University Medical Center Freiburg, University Hospital Zurich
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Awards: Computer-based analysis of early fibrosing lung disease, Enhancing the characterisation of early respiratory disease to improve population health, G6240673739, G8083610719, Enhancing the characterisation of early respiratory disease to improve population health, Computer-based analysis of early fibrosing lung disease
OpenAlex ID: W7165863234
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