Introduction: Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) is generally considered a manifestation of systemic immune dysregulation and occurs in 10-20% of people with Common Variable immunodeficiency Disorders (CVID). There is a lack of evidence-based guidelines for the diagnosis and management of GLILD due to the heterogeneity of the disease.

Aim: To systematically review the diagnostic tests used when assessing patients with CVID for possible GLILD and to evaluate their utility and risks.

Methods: The data sources used were EMBASE, MEDLINE, PubMed and Cochrane databases. Papers reporting information on the diagnosis of ILD in patients with CVID were included.

Results: In total 51 studies were included. Radiology was the investigation most commonly used during the process of diagnosis. HRCT was the most reported test, as abnormal radiology usually first raised suspicion of GLILD. Lung biopsy was used in 38 (75%) of studies, and surgical lung biopsy had more conclusive results compared to trans-bronchial biopsy (TBB). Analysis of broncho-alveolar lavage was reported in 21 (41%) studies, primarily to exclude infection. Pulmonary function tests, most commonly gas transfer was widely used during the diagnostic prosses. However, results vary from normal to severely impaired, typically with restrictive patterns and reduced gas transfer.

Conclusion: Consensus diagnostic criteria are urgently required to support accurate assessment and monitoring in GLILD. ESID and the ERS e-GLILDnet CRC have initiated a diagnostic and management guideline through international collaboration.The guideline will promote collaboration and disease management, and reduce unwarranted variation in care.