Abstract

Common variable immunodeficiency (CVID) is a form of primary immunodeficiency characterized by a low serum level of immunoglobulins and recurrent infections. Approximately 8-22% of patients with CVID develop lymphocytic granulomatous interstitial lung disease (GLILD). The confirmation of diagnosis is histological and mostly performed on surgical lung biopsy (SLB). According to new guidelines, transbronchial lung cryobiopsy (TBLC) in ILDs offers important diagnostic information with a shorter hospital stays and less major adverse events in comparison with SLB. The aim of our retrospective observational study is to evaluate the role of TBLC in the diagnostic work-up of a cohort of GLILD patients.The patients who received a multidisciplinary diagnosis of GLILD owing to a histological diagnosis obtained by TBLC in the period from September 2011 to July 2022 were retrospectively identified from our database. Data on clinical, laboratory and broncho-alveolar lavage fluid parameters, pulmonary function tests, radiological and pathological findings were retrieved from the registries. Samples obtained by TBLC were all considered adequate and histological features characteristic for GLILD were found in all nine patients. Pneumothorax was documented in two patient (22%), but none of them required chest drainage. Minor bleeding was reported in one patient (11%) during the procedure. This study demonstrates that transbronchial cryobiopsy is a safe and minimally invasive approach for characterizing GLILD in CVID patients, providing a high diagnostic confidence and low rate of complications.