Demographic, Clinical, and Immunological Features in Combined Immunodeficiency Patients with and Without Pulmonary Complications: A Retrospective Multicenter Study from Iran

Abstract

Purpose: Combined immunodeficiency (CID) is characterized by profound defects in the development and function of both B and T cells. We aimed to investigate clinical and immunological phenotype in CID patients with and without pulmonary complications.Methods: This retrospective study was performed on patients with established diagnosis of CID registered between 2009 and 2020. Patients were divided into two groups based on the development of pulmonary complications, and their demographic, clinical, and laboratory characteristics were compared. All data were analyzed by SPSS software, and a P-value <0.05 was considered as a significant difference.Results: 146 patients [56.8% male and 43.2% females] were enrolled in the study and divided into two groups of patients with (n=88) and without (n=58) pulmonary complications. In patients with pulmonary complications, oral candidiasis, failure to thrive, and otitis media, while in the other group, anemia, autoimmunity, rheumatologic disorders, and skin lesions had higher frequency, although not significant. Thoracic high resolution computed tomographies (HRCTs), available in 54.5% of patients with pulmonary complications, were compatible with pneumonia (39.8%), bronchiectasis (12.5%), pulmonary nodules (3.4%), atelectasis (1.1%), interstitial lung disease (1.1%), and pneumothorax (1.1%). Patients with pulmonary complications had lower number of T CD4+ but higher levels of CD8+ cells compared to patients without pulmonary complications (p=0.012 and p=0.005, respectively). The mortality rate was higher in patients with pulmonary complications compared to the other group (11.4% vs. 6.9%, p=0.597).Conclusion: Respiratory disorders in CID are common and require early periodic monitoring by respiratory tests and HRCT to avoid irreversible injuries.