Autoimmunity in cystic fibrosis: significance and clinical implications

Abstract

Anti-neutrophil cytoplasmic antibodies specific for bactericidal/permeability-increasing protein (BPI-ANCA) are frequently present in cystic fibrosis patients. These autoantibodies are believed to develop in response to infection and colonization by Pseudomonas aeruginosa. Development of BPI-ANCA has been shown to correlate with the severity of lung infection and poor prognosis in cystic fibrosis patients.

Keywords

• Anti-neutrophil cytoplasmic antibodies, autoimmune disease, inflammation, bactericidal/permeability-increasing protein, cystic fibrosis, Pseudomonas aeruginosa