Clinical significance of BPI-ANCA in patients with cystic fibrosis: a single center prospective study.

Recurrent pulmonary exacerbation due to infection and inflammation remain the major cause of mortality and morbidity in patients with cystic fibrosis (CF). Increased levels of BPI-ANCA have been linked to Pseudomonas colonization and pulmonary exacerbations in patients with CF. The majority of these studies were done in Europe, and it is unclear whether similar findings are true in CF patients who lives in United States. In our single center study of 47 patients with CF, the prevalence of BPI-ANCA was 19% at baseline and 15% at annual follow-up visit. Overall, there were no statistical differences noted in FEV1 and frequency of pulmonary exacerbations in CF patients who were positive for BPI-ANCA compared to those who were negative for BPI-ANCA. The role of BPI-ANCA in patients with CF still remains unclear.

Prevalence of Bactericidal/Permeability-Increasing Protein Autoantibodies in Cystic Fibrosis Patients: Systematic Review and Meta-Analysis.

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.

When to suspect atypical cystic fibrosis.

Not all patients with cystic fibrosis have abnormal sweat chloride levels, severe lung disease, or failure to thrive. These 2 cases remind us to think "outside the box".

Treatment of allergic bronchopulmonary aspergillosis (ABPA) in CF with anti-IgE antibody (omalizumab).

Allergic bronchopulmonary aspergillosis (ABPA) results from IgE induced pulmonary response to aspergillus species. Recognition and management of ABPA is challenging in cystic fibrosis (CF) patients because changes in symptoms, lung function and chest radiograph are similar to that seen in CF related pulmonary infection. Standard therapy for ABPA includes systemic steroids and adjunctive use of antifungal agents. Little has been published regarding the use of monoclonal anti-IgE antibody in those with ABPA. We report a CF patient with her third exacerbation of ABPA who was treated with monoclonal anti-IgE (omalizumab) antibody; she had unfavorable side effects with prednisone therapy. This therapy resulted in improvement of pulmonary symptoms and lung function not achieved with antibiotics or prednisone alone.

Bronchial anomalies in VACTERL association.

VACTERL association is an acronym made of associated defects including vertebral anomalies, anal atresia, cardiac, tracheal-esophageal fistula, and renal/radial limb anomalies. Tracheal bronchus is a condition characterized by ectopic location of the right upper lobe bronchus at the mid to distal trachea. This condition is associated with congenital anomalies and has been reported in one previous case of VACTERL. We report another infant with VACTERL presenting with respiratory complications due to presence of tracheal bronchus. She also had a narrowed segment of her right main stem bronchus.