ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS IN PATIENTS WITH CYSTIC-FIBROSIS

In order to determine the incidence of allergic bronchopulmonary asper gillosis (ABPA) in patients with cystic fibrosis (CF), we reviewed the records of 236 patients followed up at the Duke CF Center. Sixty pati ents (25 percent) had colonies of Aspergillus fumigatus. These patient s were older and had more severe disease as assessed by lower Shwachma n-Kulczycki (S-K) scores than the patients who did not have evidence o f A fumigatus. In 15 of the patients with A fumigatus (6.5 percent of the total population), the diagnosis was ABPA. Age and S-K scores were not significantly different from those of the patients with A fumigat us without ABPA. Diagnostic features of the affected patients included wheezing refractory to bronchodilator therapy, persistent pulmonary i nfiltrates, peripheral eosinophilia, positive skin reactivity to an A fumigatus antigen and elevated total serum IgE levels. Steroid therapy was started for all patients, and clinical improvement was noted with in 1 month as evidenced by decreased symptoms and weight gain. Chest x -ray films usually showed improvement. Vital capacity improved in all but two patients. Total IgE did not consistently decrease in response to therapy. Although the diagnosis of ABPA may be difficult to establi sh, ABPA. commonly is associated with CF. Most patients respond to ste roid therapy; however, the effect of therapy on the course of the dise ase is difficult to assess.