Allergic bronchopulmonary aspergillosis in cystic fibrosis

Reported prevalence, regional distribution, and patient characteristics

David E. Geller, Haley Kaplowitz, Michael J. Light, Andrew Colin

Research output: Contribution to journalArticle

150 Citations (Scopus)

Abstract

Objectives: Using the large database from the Epidemiologic Study of Cystic Fibrosis (ESCF), the objectives of this study were to (1) estimate the reported prevalence of allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis (CF); (2) compare reported prevalence rates across geographic regions; (3) compare reported prevalence rates between patient subgroups based on demographic and disease characteristics; and (4) describe the ABPA group with regard to their sex, age, and disease severity. Study design: All patients ≥ 5 years of age enrolled in ESCF between December 1993 and May 1996 were eligible. Criteria for the diagnosis of ABPA were defined by the ESCF guidelines. Prevalence rates for ABPA were calculated, and potential risk factors for the diagnosis of ABPA were analyzed, including sex, age, pulmonary function, diagnosis of asthma, presence of wheeze, and positive respiratory culture for Pseudomonas. Results: There were 14,210 eligible patients enrolled in ESCF during this period, and ABPA was diagnosed in 281 patients (2%). Regional prevalence varied from 0.9% in the Southwest to 4.0% in the West. Increased prevalence rates occurred in female patients, the adolescent age group, and subjects with lower lung function, wheeze, asthma, and positive Pseudomonas cultures. Although most ABPA patients had evidence of airway obstruction, 10% had an FEV1 of > 100% of predicted. The rates of wheeze (17%) and asthma (30%) were lower than expected in the ABPA group. Conclusions: This observational study found a reported prevalence rate of ABPA of 2% of CF patients in a large database. This rate was lower than the 5 to 15% rate reported in smaller studies, suggesting that ABPA is underdiagnosed in the CF population. There was wide regional variation in reported prevalence rates, which is unexplained at this time. The characteristics of the patients with ABPA and the epidemiologic risk factors for diagnosis of ABPA were described. Simplified diagnostic criteria were adapted for ESCF with the intent of increasing awareness of ABPA among the participants in this study.

Original languageEnglish
Pages (from-to)639-646
Number of pages8
JournalChest
Volume116
Issue number3
DOIs
StatePublished - Sep 21 1999
Externally publishedYes

Fingerprint

Allergic Bronchopulmonary Aspergillosis
Cystic Fibrosis
Epidemiologic Studies
Asthma
Pseudomonas
Epidemiologic Factors
Databases
Lung
Airway Obstruction

Keywords

  • Allergic bronchopulmonary aspergillosis
  • Cystic fibrosis
  • Epidemiology

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Allergic bronchopulmonary aspergillosis in cystic fibrosis : Reported prevalence, regional distribution, and patient characteristics. / Geller, David E.; Kaplowitz, Haley; Light, Michael J.; Colin, Andrew.

In: Chest, Vol. 116, No. 3, 21.09.1999, p. 639-646.

Research output: Contribution to journalArticle

Geller, David E. ; Kaplowitz, Haley ; Light, Michael J. ; Colin, Andrew. / Allergic bronchopulmonary aspergillosis in cystic fibrosis : Reported prevalence, regional distribution, and patient characteristics. In: Chest. 1999 ; Vol. 116, No. 3. pp. 639-646.
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abstract = "Objectives: Using the large database from the Epidemiologic Study of Cystic Fibrosis (ESCF), the objectives of this study were to (1) estimate the reported prevalence of allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis (CF); (2) compare reported prevalence rates across geographic regions; (3) compare reported prevalence rates between patient subgroups based on demographic and disease characteristics; and (4) describe the ABPA group with regard to their sex, age, and disease severity. Study design: All patients ≥ 5 years of age enrolled in ESCF between December 1993 and May 1996 were eligible. Criteria for the diagnosis of ABPA were defined by the ESCF guidelines. Prevalence rates for ABPA were calculated, and potential risk factors for the diagnosis of ABPA were analyzed, including sex, age, pulmonary function, diagnosis of asthma, presence of wheeze, and positive respiratory culture for Pseudomonas. Results: There were 14,210 eligible patients enrolled in ESCF during this period, and ABPA was diagnosed in 281 patients (2{\%}). Regional prevalence varied from 0.9{\%} in the Southwest to 4.0{\%} in the West. Increased prevalence rates occurred in female patients, the adolescent age group, and subjects with lower lung function, wheeze, asthma, and positive Pseudomonas cultures. Although most ABPA patients had evidence of airway obstruction, 10{\%} had an FEV1 of > 100{\%} of predicted. The rates of wheeze (17{\%}) and asthma (30{\%}) were lower than expected in the ABPA group. Conclusions: This observational study found a reported prevalence rate of ABPA of 2{\%} of CF patients in a large database. This rate was lower than the 5 to 15{\%} rate reported in smaller studies, suggesting that ABPA is underdiagnosed in the CF population. There was wide regional variation in reported prevalence rates, which is unexplained at this time. The characteristics of the patients with ABPA and the epidemiologic risk factors for diagnosis of ABPA were described. Simplified diagnostic criteria were adapted for ESCF with the intent of increasing awareness of ABPA among the participants in this study.",
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