Clinical trial NCT00585364: Mechanisms of Immune Tolerance and Inflammation in Patients With Cystic Fibrosis With ABPA
Official title: SCCOR in Host Factors in Chronic Lung Diseases: Mechanisms of Immune Tolerance and Inflammation in Allergic Bronchopulmonary Aspergillosis (ABPA) in Patients With Cystic Fibrosis
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Cystic Fibrosis
Allergic Bronchopulmonary Aspergillosis
Keywords: Allergic Bronchopulmonary Aspergillosis
Aspergillus fumigatus
cytokine expression
Lead sponsor: University of Pittsburgh
Sponsor class: OTHER
Collaborators: name: National Heart, Lung, and Blood Institute (NHLBI)
First posted: 2008-01-03
Enrollment: 79
Sex: ALL
Minimum age (years): 6
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Observational model: CASE_CONTROL
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Male and female subjects with CF who have A. fumigatus in cultures of airway flora and receive clinical care at the Antonio J. and Janet Palumbo Cystic Fibrosis Center at Children's Hospital of Pittsburgh. Age (± 1 year) and sex matched healthy, non CF controls will also be recruited.

Brief summary: The goal of this study is to identify the immunological factors that influence a patient's response to the presence of the fungus Aspergillus fumigatus (A. fumigatus) in the lungs. In patients with cystic fibrosis (CF), this fungus is not known to cause damage to the lungs, but some patients respond with an allergic reaction that may cause wheeze, cough, or difficulty breathing. Approximately 230 patients will be enrolled with an additional 60 people who do not have CF and who do not have a history of asthma to serve as a comparison group.

Detailed description: In this cohort the investigators will study blood T-cell responses to Aspergillus antigens to determine if patients with ABPA have greater type 2 responses as measured by IL-13 compared to CF or non-CF controls.

Eligibility criteria: Inclusion Criteria:

(CF)

* diagnosis of CF
* age 6 years or older
* presence of A. fumigatus in culture of airway flora, or the presence of one or more of the diagnostic criteria for ABPA (Control)
* age and sex matched to CF population

Exclusion Criteria:

(CF)

* uncontrolled CF-related diabetes mellitus
* use of oral steroids at a dose ≥ 0.5 mg/kg/day
* history of lung transplantation
* pulmonary exacerbation as defined by requirement for use of intravenous antibiotics or need for hospitalization within the preceding 14 days.
* patients who have a diagnosis of HIV and have a CD4+ Tcell count below 500 cells/ml will be excluded (control)
* asthma

Primary outcomes:
measure: To test the hypothesis that the white blood cells of CF patients with ABPA will demonstrate increased inflammatory cytokine expression in response to binding of A. fumigatus antigens compared to white blood cells from non-ABPA patients.
timeFrame: baseline, 6 month follow-up, ABPA exacerbation
Secondary outcomes:
measure: To test the hypothesis that T cells from CF patients with ABPA will have decreased adaptive regulatory function
timeFrame: baseline, 6 month follow-up, ABPA exacerbation
measure: To test the hypothesis that surface-bound TGF beta is critical for the development and maintenance of immune tolerance to A. fumigatus antigens
timeFrame: baseline, 6 month follow-up, ABPA exacerbation
Other outcomes:
Locations:
city: Pittsburgh
country: United States
facility: Children's Hospital of Pittsburgh of UPMC
state: Pennsylvania
Study officials:
affiliation: University of Pittsburgh
name: Jay K Kolls, MD
role: PRINCIPAL_INVESTIGATOR
References: