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. 2023 Oct 24;13(1):18138.
doi: 10.1038/s41598-023-45273-2.

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

Affiliations

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

Kenneth Iwuji et al. Sci Rep. .

Abstract

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.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Figure 1
Figure 1
Distribution of age at baseline given BPI-ANCA status. Group differences tested via Independent Student’s t-test, α = 0.05; p = 0.261.
Figure 2
Figure 2
Distribution of age at follow-up given BPI-ANCA status. Group differences tested via Independent Student’s t-test, α = 0.05; p = 0.288.
Figure 3
Figure 3
Prevalence of Pseudomonas colonization by BPI-ANCA status at baseline. Group differences tested via Fisher’s exact test, α = 0.05; p = 0.035.
Figure 4
Figure 4
Prevalence of Pseudomonas colonization by BPI-ANCA status at follow-up. Group differences tested via Fisher’s exact test, α = 0.05; p = 0.163.
Figure 5
Figure 5
Distribution of FEV1 percent predicted by BPI-ANCA status at baseline. Group differences tested via Wilcoxon rank-sum test, α = 0.05; p = 0.383.
Figure 6
Figure 6
Distribution of FEV1 percent predicted by BPI-ANCA status at follow-up. Group differences tested via Wilcoxon rank-sum test, α = 0.05; p = 0.411.

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