[Pulmonary symptoms of primary immunodeficiency diseases].

OBJECTIVE

Patients who lack major components of the immune system carry an increased risk for severe and recurrent pulmonary infections at those respiratory sites were the deficient component would, in the normal state, have its greatest concentration. We report different pulmonary manifestations in pediatric patients with primary immunodeficiency disease (PID).

PATIENTS AND METHODS

We studied 44 children younger than 14 years old, who were diagnosed of PID in our pediatric department between January 1990 and May 1996.

RESULTS

Antibody deficiencies were the most frequent disorders (27/44; 61.3%) followed by PID associated with or secondary to other disorders (10/44; 22.7%) and defects of phagocyte function (5/44; 11.3%). Twenty-seven patients (61.3%) showed relevant pulmonary manifestations that required assistance in the division of pediatric pulmonology. Bronchial responsiveness was seen in 17/27, 11/27 had recurrent pneumonias with development of bronchiectasis in 7/27. Opportunistic or severe pneumonias leading to acute respiratory failure were diagnosed in 9/27. Necrotizing pneumonias leading to development of pneumatoceles, cavities or abscesses was seen in 3/27 with the same rate for lymphoid interstitial pneumonia. Respiratory symptoms were the first manifestations of PID in 19/27 (70.3%).

CONCLUSIONS

The findings of the study emphasize the responsibility of the pediatric pulmonologists in avoiding the delayed diagnosis of PID since the prognosis depends on the precocity of diagnosis.