Demographic, Clinical, and Immunological features in Combined Immunodeficiency Patients with Pulmonary Complications: A Retrospective Multicenter Study from Iran

Abstract

Background: 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, who had available thoracic computed tomography scan in their medical records. 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: 53 patients [56.6% male and 43.4% females] were enrolled in the study and divided into two groups of patients with (n = 43) and without (n = 10) pulmonary abnormality in the HRCT. In patients with pulmonary complications, skin lesions, failure to thrive, and autoimmunity, were three top high provenances, and anemia was significantly presented in patients with complicated HRCT. Findings of thoracic high resolution computed topographies (HRCTs) included pneumonia (n = 15, 28.3%), bronchiectasis (n = 10, 18.9%), pulmonary nodules (n = 1, 1.9%), atelectasis (n = 2, 3.8%), interstitial lung disease (n = 2, 3.8%). Dead patients had significantly lower level of platelet (183000 cell/µL, P-value = 0.031), IgG (501 mg/dl, P-value = 0.037) and IgE (1.5 IU/ml, P-value = 0.046) compare to alive patients. The mortality rate was higher in patients with pulmonary complications compared to the other group (18.9% vs. 1.9%, p = 0.667).Conclusion: Respiratory disorders in CID are common and require early periodic monitoring by respiratory tests and HRCT to avoid irreversible injuries.