Respiratory system evaluation of adult primary immunodeficiency patients: a tertiary care center experience

• DOI: 10.2478/ahem-2024-0018
• Publication Date: 2025-02-01T20:22:23Z
• Authors: Saltuk Buğra Kaya; Mehmet Erdem Çakmak; Özge Can Bostan; Ebru Damadoğlu; Gül Karakaya; Ali Fuat Kalyoncu; Saliha Esenboğa; Deniz Çağdaş Ayvaz; İlhan Tezcan
• Keywords: Common Variable Immunodeficiency; Primary Immunodeficiency; Restrictive lung disease; Chronic Cough

Abstract Introduction Primary immunodeficiencies (PIDs) are rare diseases in which chronic pulmonary common. Chronic complications affect the long-term survival of these patients. The aim this study was to evaluate accompanying lung and respiratory functions adult PID patients Turkish population. Materials Methods Patients’ files who applied immunology clinic between 2015 2020 were evaluated retrospectively. system by physical examination, if necessary, computed tomography, chest radiography, function test (PFT) performed. diagnosis PIDs based on European Society Immunodeficiency’s (ESID) criteria. Results A total 186 included study. median age 38 years. distribution order frequency is: Common Variable Immunodeficiency (CVID) (47.8%), Severe Combined (SCID) (22.6%), Selective IgA deficiency (SIgAD) (10.8%), X-Linked Agammaglobulinemia (XLA) (10.2%), Granulomatous Disease (CGD) (8.6%). most common findings radiology bronchiectasis (37.1%), parenchymal nodule (32.8%), ground glass opacity (31.2%), lymphadenopathy (24.7%), fibrotic changes (24.8%), reticular opacities (23.7%) bronchial wall thickening (23.1%). PFT’s results lower with CGD. Bronchiectasis asthma (22%), tuberculosis (9.7%) complications. Conclusion We think that other complications, especially bronchiectasis, is higher PIDs, patient management poor as there no guidelines for follow-up, diagnosis, treatment