Exploring noninfectious radiological lung findings in adult patients with primary immunodeficiency diseases

Abstract

Primary immunodeficiency diseases (PIDs) show different patterns of airway involvement, particularly bronchiectasis; however, comparative studies of radiologic manifestations in patients with PIDs are scarce. Hence, the aim of this study to investigate radiologic lung findings in adult patients with PIDs and evaluate the possible relationship between clinical and immunologic features and respiratory function in these patients. In this study, the demographic and clinical characteristics, serum immunoglobulins (Ig), lymphocyte subgroups, high-resolution computed tomography (HRCT), and pulmonary function tests (PFTs) of 116 adult patients with PID were evaluated and those with and without abnormal HRCT were compared. The median age was 40 (28–48) years, and there were 51 (44%) females. Abnormal findings were detected in 55.2% of the HRCTs, but the most common findings were bronchiectasis (30.2%), bilateral involvement (73.5%), and lower lobe predominance. The median age and age of diagnosis were higher in those with HRCT findings. The obstructive pattern was the most common found in the PFTs. Forced vital capacity, maximal mid-expiratory flow at 25–75%, immunoglobulin G (IgG), immunoglobulin A (IgA), immunoglobulin M (IgM), cluster of differentiation (CD)4+ T cell, CD4+/CD8+ ratio, and class-switched memory B (cSMB) cell levels were significantly lower, whereas mortality was higher. Noninfectious pulmonary complications are among the important causes of morbidity and mortality in PID that could result in chronic lung disease despite adequate Ig therapy. Considering the extra radiation dose of HRCT, clinical findings and immunological and PFT parameters accompanying radiological features may be helpful in predicting the diagnosis; it may also be useful in determining additional treatment modalities and reducing mortality.