PREVALENCE OF BACTERICIDAL/PERMEABILITY-INCREASING PROTEIN-AUTOANTIBODIES IN CYSTIC FIBROSIS PATIENTS: SYSTEMATIC REVIEW AND META-ANALYSIS

Abstract

SESSION TITLE: Tuesday Abstract Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM PURPOSE: There have been varying reports on the prevalence of anti-neutrophil 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 I2 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 (I2 = 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. CLINICAL IMPLICATIONS: Cystic fibrosis (CF) is a multisystem life-limiting autosomal recessive disease that primarily affects Caucasians.According to the cystic fibrosis registry, over 30,000 people in United States are living with CF, and more than 1,000 new cases are diagnosed each year.Despite being a multisystem disorder, progressive deterioration of pulmonary function from chronic airway inflammation and infection, particularly with Pseudomonas aeruginosa, still remains the major cause of mortality and morbidity in patients with CF.1 Several studies have reported a varying prevalence of ANCA in CF patients, especially those with BPI-ANCA. Anti-bactericidal/permeability-increasing protein specificity has been identified in a variety of diseases, such as cystic fibrosis, inflammatory bowel diseases, vasculitis, rheumatoid arthritis, and primary sclerosing cholangitis.3,4,5 The occurrence of BPI-ANCA in CF patients is seen after colonization by more virulent strains of P.aeruginosa.6 In the last decade, several single center studies have reported varying prevalence rates of BPI-ANCA in CF patients and its link to a poor prognosis by progressive lung function deterioration.3,6,9,10 The aim of this study was to systematically review the existing literature that presents estimates for the prevalence of BPI-ANCA in patients with CF. DISCLOSURES: No relevant relationships by Sharan Bijlani, source=Web Response No relevant relationships by Kenneth Iwuji, source=Web Response No relevant relationships by Kenneth Nugent, source=Web Response No relevant relationships by Ximena Solis,