Chest
Volume 105, Issue 1, January 1994, Pages 32-36
Journal home page for Chest

Clinical Investigations: Pediatrics
Allergic Bronchopulmonary Aspergillosis in Patients With Cystic Fibrosis

https://doi.org/10.1378/chest.105.1.32Get rights and content

In order to determine the incidence of allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis (CF), we reviewed the records of 236 patients followed up at the Duke CF Center. Sixty patients (25 percent) had colonies of Aspergillus fumigatus. These patients were older and had more severe disease as assessed by lower Shwachman-Kulczycki (S-K) scores than the patients who did not have evidence of 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 fumigatus without ABPA. Diagnostic features of the affected patients included wheezing refractory to bronchodilator therapy, persistent pulmonary infiltrates, 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 within 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 establish, ABPA commonly is associated with CF. Most patients respond to steroid therapy; however, the effect of therapy on the course of the disease is difficult to assess.

Section snippets

Patients and Methods

During a 6-month period in 1985, we reviewed records of 236 patients with CF who were diagnosed and followed up at the Duke CF center. These patients had blood counts, chest x-ray films, and sputum or throat swab specimens submitted for culture as part of their routine care. The sputum specimens were plated on routine blood agar nonenhanced media. Spirometric testing was performed on patients old enough to cooperate and forced vital capacity, FEV1, and mean forced expiratory flow during the

Results

The patients ranged in age from 1 to 41 years, with a mean age of 14.5 years. All patients were white; there were 11 male and only 4 female patients, with a ratio of 2.7:1. Their Shwachman-Kulczycki (S-K) scores13 ranged from 35 to 100 with a mean of 78.5. Sixty patients (25 percent of the population) were found to have colonies of A fumigatus, as evidenced by growth on the sputum culture. These patients were significantly older (p < 0.0002) and had more severe disease, as assessed by a lower

Discussion

Cystic fibrosis is an autosomal recessive disease involving dysfunction of the exocrine glands. One major target organ is the lung where the presence of thick tenacious secretions favor the growth of certain microorganisms, especially Pseudomonas aeruginosa and Staphylococcus aureus. The secretory products of these microorganisms along with the inflammatory response initiated by their presence result in lung damage involving predominantly the upper lobe.

Allergic bronchopulmonary aspergillosis

ACKNOWLEDGMENT

The help of David F. Merten, M.D., in reviewing the radiologic findings is greatly appreciated.

References (26)

  • BasischJ.E. et al.

    Allergic bronchopulmonary aspergillosis in corticosteroid-dependent asthmatics

    J Allergy Clin Immunol

    (1981)
  • MearnsM. et al.

    Transient pulmonary infiltrations in cystic fibrosis due to allergic aspergillosis

    Thorax

    (1965)
  • BattenJ.C.

    Allergic Aspergillus in cystic fibrosis

    Mod Probi Pediatr

    (1967)
  • Cited by (127)

    • The presence of Aspergillus fumigatus is associated with worse respiratory quality of life in cystic fibrosis

      2020, Journal of Cystic Fibrosis
      Citation Excerpt :

      To our knowledge, our study is the first investigation of the relationship between Af and patient-reported outcomes in a general CF population. Lung function decline, pulmonary exacerbations, and radiographic findings of lung disease have been the outcomes of previous studies examining the clinical effects of Aspergillus species [4,24–29]. Aaron et al. conducted a randomized, placebo-controlled trial of itraconazole for chronic Af in CF patients which did not find a difference in change in respiratory domain score of CFQ-R and other clinical endpoints over 24-week treatment period.

    • What is the clinical significance of filamentous fungi positive sputum cultures in patients with cystic fibrosis?

      2013, Journal of Cystic Fibrosis
      Citation Excerpt :

      Two older retrospective studies in German [12] and French children [10] found no associations between AC and clinical parameters like FEV1, Shwachman–Kulczycki (S–K) scores and radiological scores of lung disease. In contrast to these studies, two reports from US CF centres reported lower S–K and higher chest radiograph scores in patients who had Aspergillus spp. isolated from the airways [9,11]. Both these studies assigned patients to the Aspergillus spp. group on the basis of a single isolate which does not constitute colonisation and these studies are not directly comparable to the more recent studies stipulating repeated isolation for a diagnosis of AC.

    • Aspergillus fumigatus colonization in cystic fibrosis: Implications for lung function?

      2011, Clinical Microbiology and Infection
      Citation Excerpt :

      Filamentous fungi such as Aspergillus fumigatus are also commonly found in respiratory secretions of patients. Reported prevalence rates of colonization in patients with CF vary, ranging from 6% to 58% [2–11]. The variation in the reported prevalence has been hypothesized to be attributable to the age of patients and climatic conditions [8].

    View all citing articles on Scopus
    View full text