Background Primary immunodeficiencies are a heterogeneous group of genetic diseases caused by one or more abnormalities in the immune system. Although pulmonary complications are common in patients with primary immunodeficiency diseases, these complications contribute significantly to morbidity and mortality. Aim The aim of our study was to evaluate the distribution of the features of pulmonary radiological involvement and demographic findings in this patient group. Materials and methods The files of patients who were treated and followed up with the diagnosis of primary immunodeficiency between 2014 and 2021 were analyzed retrospectively. Demographic data, symptoms, additional diseases, and computed tomography findings of the patients were recorded. Results The mean age of 32 cases was 37.34±13.54 (20-69) and the age of diagnosis was 28.90±15.75 (1-62). Twenty of the cases were male and 10 were female. The most common symptom was diarrhea with 53.1% and cough with 34.4%. The most common radiological finding is bronchiectasis in 75% of cases. Twenty-one (65.6%) of the cases had recurrent pneumonia before diagnosis and no pneumonia was observed after intravenous ımmunoglobulin replacement therapy. Three of the cases (9.4%) died during the follow-up. Conclusions Primary immunodeficiency should be considered in patients with bronchiectasis and a history of recurrent pneumonia, and further investigations should be performed. Early diagnosis of patients with primary immunodeficiency is very important for the early detection and treatment of malignancy and the interstitial lung disease that may develop.
Keywords: bronchiectasis; computed tomography; interstitial lung; primary immunodeficiency; prımary antibody deficiency.
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