BPI-ANCA Provides Additional Clinical Information to Anti-PseudomonasSerology: Results from a Cohort of 117 Swedish Cystic Fibrosis Patients

Abstract

Patients with cystic fibrosis (CF) colonized withPseudomonas aeruginosa(P. aeruginosa) have worse prognosis compared with patients who are not. BPI-ANCA is an anti-neutrophil cytoplasmic antibody against BPI (bactericidal/permeability increasing protein) correlating withP. aeruginosacolonization and adverse long time prognosis. Whether it provides additional information as compared to standard anti-P. aeruginosaserology tests is not known. 117 nontransplanted CF patients at the CF centre in Lund, Sweden, were followed prospectively for ten years. Bacterial colonisation was classified according to the Leeds criteria. IgA BPI-ANCA was compared with assays for antibodies against alkaline protease (AP), Elastase (ELA), and Exotoxin A (ExoA). Lung function and patient outcome, alive, lung transplanted, or dead, were registered. BPI-ANCA showed the highest correlation with lung function impairment with anr-value of 0.44. Forty-eight of the 117 patients were chronically colonized withP. aeruginosa. Twenty of these patients experienced an adverse outcome. Receiver operator curve (ROC) analysis revealed that this could be predicted by BPI-ANCA (AUC = 0.77), (p=0.002) to a better degree compared with serology tests. BPI-ANCA correlates better with lung function impairment and long time prognosis than anti-P. aeruginosaserology and has similar ability to identify patients with chronicP. aeruginosa.