Autoimmunity in cystic fibrosis: significance and clinical implications

Kenneth Iwuji MD, Sharan Bijlani BA, Rose Izuchi MD, David Sotello MD


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

Article citation: Iwuji K, Bijlani S, Izuchi R, Sotello D. Autoimmunity in cystic fibrosis: significance and clinical implications. The Southwest Respiratory and Critical Care Chronicles. 2018;6(23):48–50
From: Department of Internal Medicine at Texas Tech University Health Sciences Center, Lubbock, TX
Submitted: 2/14/2018
Accepted: 3/23/2018
Reviewer: Adaobi Kanu MD, John Pixley MD
Conflicts of interest: None
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