Allergic Bronchopulmonary Aspergillosis in Patients With Cystic Fibrosis*

In orderto determine the incidence ofallergic bronchopulmonary aspei llosis (ABPA) in patients with cyslic fibrosis (CF), we reviewed the records of 236 patients followed up at the Duke CF Center. Sixty patients (25 percent) had colonies ofAspergiliusjismigatus. Thesepafients were older and had more severe disease as assessed by lower Shwachman-Kulczycld (S-K) scores than the patients who did not have evidence ofAfumigatus. In 15 of the patients with A jismigatus (6.5 percent of the total population), the diagnosis was ABPA. Age and S-K scores were not significandy different from those ofthe patients withAfumigatus without ABPA. Diagnostic features ofthe affected patients included wheezing refractory to bronchodilator therapy, persistent puhnonaiy infiltrates, peripheral eosinopbilia, positive skin reactivity to an AfumigatUs antigen and elevated total serum IgElevels. Steroidtherapywas started for all patients, and clinical improvement was noted within 1 month as evidenced by decreased symptoms and weight gain. Chest x-ray films usually showed improvement Vital cap 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 establish, ABPA commonly is associated with CF. Most patients respond to sternidtherapy;however,the effectoftherapyon the course of the disease is difficult to assess. (Chest 1994; 105:32-36)