Mechanisms of Immune Tolerance and Inflammation in Patients With Cystic Fibrosis With ABPA



Status:Completed
Conditions:Allergy, Pulmonary
Therapuetic Areas:Otolaryngology, Pulmonary / Respiratory Diseases
Healthy:No
Age Range:6 - Any
Updated:4/21/2016
Start Date:March 2005
End Date:December 2010

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SCCOR in Host Factors in Chronic Lung Diseases: Mechanisms of Immune Tolerance and Inflammation in Allergic Bronchopulmonary Aspergillosis (ABPA) in Patients With Cystic Fibrosis

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.

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.

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
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Pittsburgh, Pennsylvania 15224
412-692-5325
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