THE CLINICAL SPECTRUM OF RESPIRATORY DISEASES IN PATIENTS WITH PRIMARY ANTIBODY DEFICIENCY

Primary Humoral Immunodeficiencies (PHID) are currently increasingly being recognized. Patients with PHID frequently show respiratory complications. The objectives of the study is to determine the clinical spectrum of respiratory diseases in patients with PHID. We extracted data from the clinical files of patients with PHID, diagnosed according to WHO criteria. We encountered 125 patients (84 males), with the diagnosis of primary antibody deficiency including common-variable immunodeficiency (64 pts), x-linked agammaglobulinemia (29 pts), IgA deficiency (20 pts), IgG-subclass deficiency (8 pts), and hyper-IgM syndrome (4 pts). The mean age of the patients at the time of study was 11 years. In the evolution of their disease, 92 cases (73.6%) developed upper respiratory tract infections, among which acute otitis media (68 pts, 54.4%), sinusitis (61 pts, 48.8%), and pharyngitis (12 pts, 10.4%) were found to be the most frequent. Among the lower respiratory tract infections, pneumonia was the most common occurance (91 pts, 72.8%). The other lower respiratory tract complications were: bronchiectasis (22 pts, 17.6%), bronchitis (8 pts), tuberculosis (6 pts), lung abscess (4 pts), and pneumocystis carinii pneumonia (2 pts). Respiratory infections constitute the most common presenting symptom of patients with primary humoral immunodeficiency. There may be some differences in the type and frequency of infections in each of these disorders.