Allergic Bronchopulmonary Aspergillosis and Cystic Fibrosis

Allergic bronchopulmonary aspergillosis is a hypersensitivity lung disease mediated by a Th2 hypersensitivity inflammatory response due to bronchial colonization with Aspergillus fumigatus. ABPA principally affects genetically susceptible asthmatic and cystic fibrosis (CF) patients. Genetic risk factors in the development of ABPA include HLA-DR/HLA-DQ associations, IL-4RA gain-of-function (GOF) single-nucleotide polymorphisms (SNPs) that increase sensitivity to IL-4 stimulation, and cystic fibrosis transmembrane conductance regulator (CFTR) mutations. Understanding the immunogenetics and immunopathogenesis of ABPA offers the opportunity for targeted treatments using biologicals modulating the Th2 inflammatory response and CFTR modulators improving the function of abnormal CFTR proteins in CF ABPA patients.