%0 Systematic Review
%T Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review.
%A Bintalib HM
%A van de Ven A
%A Jacob J
%A Davidsen JR
%A Fevang B
%A Hanitsch LG
%A Malphettes M
%A van Montfrans J
%A Maglione PJ
%A Milito C
%A Routes J
%A Warnatz K
%A Hurst JR
%J Front Immunol
%V 14
%N 0
%D 2023
%M 37223103
%F 8.786
%R 10.3389/fimmu.2023.1190235
%X 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.<BR>To systematically review use of diagnostic tests for assessing patients with CVID for possible ILD, and to evaluate their utility and risks.<BR>EMBASE, MEDLINE, PubMed and Cochrane databases were searched. Papers reporting information on the diagnosis of ILD in patients with CVID were included.<BR>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.<BR>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.<BR>https://www.crd.york.ac.uk/prospero/, identifier CRD42022276337.