Background: There have been varying reports on the prevalence of antineutrophil cytoplasmic antibodies with bactericidal/permeability-increasing protein (BPI-ANCA) specificity in cystic fibrosis (CF) patients. These autoantibodies are believed to develop in response to infection and colonization, especially with Pseudomonas aeruginosa. The aim of this review was to estimate the overall prevalence of BPI-ANCA in CF patients. Methods: We searched PubMed, Scopus, and Embase databases for studies reporting the prevalence of BPI-ANCA in CF patients. We also searched the Journal of Cystic Fibrosis and our institution's library for relevant studies on BPI-ANCA. We calculated the proportion with a 95% confidence interval (CI) to assess the prevalence of BPI-ANCA in the individual studies and then pooled the results using a random effects model. Publication bias was assessed using graphical and statistical methods. Finally, we assessed statistical heterogeneity using the I 2 test. Results: Our search yielded 12 eligible studies published between 1996 and 2015. The prevalence of BPI-ANCA in patients with CF varied from 17.9% to 83% with a pooled prevalence of 49.45% (95% CI 35. 53-63.42). No evidence of bias was found. However, there was evidence of statistically significant variation in the prevalence estimate due to heterogeneity (I 2 = 93.4%, P < 0.01). Conclusions: Given the highly varying prevalence of BPI-ANCA in patients with CF, more well-designed prospective clinical studies are needed to determine its true prevalence and clinical relevance.

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