Background

Autoantibodies to bactericidal/permeability-increasing protein (BPI), BPI-ANCA, are often present in serum of patients with cystic fibrosis (CF), and correlate to airway colonization with Pseudomonas aeruginosa. The aim of the study was to investigate if BPI-ANCA IgA is also present in the airways of CF patients, and if its presence correlates with neutrophil counts, platelets, and P. aeruginosa DNA in sputum.

Methods

BPI-ANCA IgA was quantified in serum and sputum samples from adult CF patients (n = 45) by ELISA. Sputum neutrophil counts, platelets, and platelet-neutrophil complexes were assessed by flow cytometry, and P. aeruginosa DNA was analysed with RT-PCR.

Results

Serum BPI-ANCA IgA was present in 44% of the study participants, and this group also had significantly enhanced BPI-ANCA levels in sputum compared to serum negative patients. Sputum levels of BPI-ANCA IgA correlated with P. aeruginosa DNA (r = 0.63, p = 0.0003) and platelet counts in sputum (r = 0.60, p = 0.0002).

Conclusions

BPI-ANCA is expressed in the airways of CF patients and correlates with P. aeruginosa load and platelet counts, suggesting a link to airway inflammation and mucosal immunity.

中文翻译：

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BPI-ANCA在囊性纤维化患者的气道中表达，并与血小板数量和铜绿假单胞菌定植有关。

背景

杀菌/通透性增强蛋白（BPI）的自身抗体BPI-ANCA通常存在于囊性纤维化（CF）患者的血清中，并与铜绿假单胞菌与气道定植有关。该研究的目的是调查CF患者气道中是否还存在BPI-ANCA IgA，以及其是否与痰液中的中性粒细胞计数，血小板和铜绿假单胞菌DNA相关。

方法

通过ELISA对成人CF患者（n = 45）的血清和痰液样本中的BPI-ANCA IgA进行定量。通过流式细胞术评估痰中性粒细胞计数，血小板和血小板-中性粒细胞复合物，并用RT-PCR分析铜绿假单胞菌DNA。

结果

血清BPI-ANCA IgA存在于44％的研究参与者中，并且与血清阴性患者相比，该组的痰中BPI-ANCA水平也显着提高。BPI-ANCA IgA的痰液水平与铜绿假单胞菌DNA（r = 0.63，p = 0.0003）和痰液中的血小板计数（r = 0.60，p = 0.0002）相关。

结论

BPI-ANCA在CF患者的气道中表达，并与铜绿假单胞菌载量和血小板计数相关，提示与气道炎症和粘膜免疫有关。