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Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review

Bintalib, Heba M; van de Ven, Annick; Jacob, Joseph; Davidsen, Jesper Rømhild; Fevang, Børre; Hanitsch, Leif G; Malphettes, Marion; ... Hurst, John R; + view all (2023) Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review. Frontiers in Immunology , 14 , Article 1190235. 10.3389/fimmu.2023.1190235. Green open access

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Abstract

INTRODUCTION: Common variable immunodeficiency related interstitial lung disease (CVID-ILD, also referred to as GLILD) is generally considered a manifestation of systemic immune dysregulation occurring in up to 20% of people with CVID. There is a lack of evidence-based guidelines for the diagnosis and management of CVID-ILD. AIM: To systematically review use of diagnostic tests for assessing patients with CVID for possible ILD, and to evaluate their utility and risks. METHODS: EMBASE, MEDLINE, PubMed and Cochrane databases were searched. Papers reporting information on the diagnosis of ILD in patients with CVID were included. RESULTS: 58 studies were included. Radiology was the investigation modality most commonly used. HRCT was the most reported test, as abnormal radiology often first raised suspicion of CVID-ILD. Lung biopsy was used in 42 (72%) of studies, and surgical lung biopsy had more conclusive results compared to trans-bronchial biopsy (TBB). Analysis of broncho-alveolar lavage was reported in 24 (41%) studies, primarily to exclude infection. Pulmonary function tests, most commonly gas transfer, were widely used. However, results varied from normal to severely impaired, typically with a restrictive pattern and reduced gas transfer. CONCLUSION: Consensus diagnostic criteria are urgently required to support accurate assessment and monitoring in CVID-ILD. ESID and the ERS e-GLILDnet CRC have initiated a diagnostic and management guideline through international collaboration. SYSTEMATIC REVIEW REGISTRATION: https://www.crd.york.ac.uk/prospero/, identifier CRD42022276337.

Type: Article
Title: Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review
Location: Switzerland
Open access status: An open access version is available from UCL Discovery
DOI: 10.3389/fimmu.2023.1190235
Publisher version: https://doi.org/10.3389/fimmu.2023.1190235
Language: English
Additional information: © 2023 Bintalib, van de Ven, Jacob, Davidsen, Fevang, Hanitsch, Malphettes, van Montfrans, Maglione, Milito, Routes, Warnatz and Hurst. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
Keywords: CVID, GLILD, diagnosis, interstitial lung disease, systematic review, Humans, Common Variable Immunodeficiency, Lung Diseases, Interstitial, Diagnostic Techniques and Procedures, Biopsy, Affect
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Respiratory Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/10171491
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