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

University of Pittsburgh

Status

Completed

Conditions

Allergic Bronchopulmonary Aspergillosis

Cystic Fibrosis

Study type

Observational

Funder types

Other

NIH

Identifiers

NCT00585364

P50HL084932 (U.S. NIH Grant/Contract)

SCCOR

Details and patient eligibility

About

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.

Full 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.

Enrollment

79 patients

Sex

All

Ages

6+ years old

Volunteers

Accepts Healthy Volunteers

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