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