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

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

Angaben zum Objekt

• Standort: Deutsche Nationalbibliothek Frankfurt am Main
• Umfang: Online-Ressource
• Sprache: Englisch
• Anmerkungen: Frontiers in immunology. - 14 (2023) , 1190235, ISSN: 1664-3224

Beteiligte, Orts- und Zeitangaben

• Ereignis: Veröffentlichung
• (wo): Freiburg
• (wer): Universität
• (wann): 2023
• Urheber: Bintalib, Heba M. ; Ven, Annick van de ; Jacob, Joseph ; Davidsen, Jesper Rømhild ; Fevang, Børre ; Hanitsch, Leif Gunnar ; Malphettes, Marion ; Montfrans, Joris van ; Maglione, Paul J. ; Milito, Cinzia ; Routes, John M. ; Warnatz, Klaus ; Hurst, John R.

Weitere Informationen

• DOI: 10.3389/fimmu.2023.1190235
• URN: urn:nbn:de:bsz:25-freidok-2366430
• Rechteinformation: Open Access; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Letzte Aktualisierung: 14.08.2025, 10:58 MESZ