Autoimmunity in cystic fibrosis: significance and clinical implications

  • Kenneth Iwuji
  • Sharan Bijlani
  • Rose Izuchi
  • David Sotello
Keywords: Anti-neutrophil cytoplasmic antibodies, autoimmune disease, inflammation, bactericidal/permeability-increasing protein, cystic fibrosis, Pseudomonas aeruginosa


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.


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How to Cite
Iwuji, K., Bijlani, S., Izuchi, R., & Sotello, D. (2018). Autoimmunity in cystic fibrosis: significance and clinical implications. The Southwest Respiratory and Critical Care Chronicles, 6(23), 48-50.