Beta-defensins and LL-37 in bronchoalveolar lavage fluid of patients with cystic fibrosis.
Beta-defensins and LL-37 in bronchoalveolar lavage fluid of patients with cystic fibrosis.
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DOI：
10.1016/j.jcf.2003.12.008
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发表时间：
2004-03-01
期刊：
影响因子：
--
通讯作者：
Bals, Robert
中科院分区：
文献类型：
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作者：
Chen, Christiane I-U;Schaller-Bals, Susanne;Bals, Robert
背景技术背景：抗微生物肽人β-防御素1和2（hBD-1和2）和cathelicidin LL-37/hCAP-18是呼吸道先天免疫应答的关键因素。本研究的目的是确定这些肽在轻度肺部疾病的CF患者的气道表面液中的浓度。我们测量了hBD-1，hBD-2，20例患者支气管肺泡灌洗液中LL-37（5-34岁）参与前瞻性BEAT研究结果：在研究人群的灌洗液中可以检测到所有三种肽。支气管肺泡灌洗液中炎性标志物水平的增加与LL-37/hCAP-18浓度的升高相关（总细胞计数，P = 0.006;相对中性粒细胞计数，P = 0.002）。肺功能恶化，通过MEF 25测量（25%残余用力肺活量时的最大流速），与hBD-2降低相关（P = 0.026），但增加LL-37/hCAP-18浓度（P = 0.016）。结论：数据表明抗微生物肽的浓度与CF肺病的严重程度相关：LL-37/hCAP-18水平与支气管炎症相关，因此与疾病严重程度相关，而晚期肺病中β-防御素水平降低可能导致局部宿主防御的继发性缺陷。
BACKGROUND: The antimicrobial peptides human beta-defensin 1 and 2 (hBD-1 and 2) and the cathelicidin LL-37/hCAP-18 are key factors in innate immune responses of the respiratory tract. The aim of this study was to determine the concentrations of these peptides in airway surface fluid of CF patients with mild lung disease.METHODS: We measured the concentrations of hBD-1, hBD-2, and LL-37 in bronchoalveolar lavage fluid of 20 patients (5-34 years) participating in the prospective BEAT-study (bronchoalveolar lavage for the evaluation of anti-inflammatory treatment) using an immuno-dot blot-assay.RESULTS: All three peptides could be detected in lavage fluid of the study population. Increased levels of inflammatory markers in bronchoalveolar lavage fluid were associated with elevated concentrations of LL-37/hCAP-18 (total cell count, P = 0.006; relative neutrophil count, P = 0.002). Deterioration of lung function, measured by MEF25 (maximal flow rate at 25% of residual forced vital capacity), correlated with decreased hBD-2 (P = 0.026), but increased LL-37/hCAP-18 concentrations (P = 0.016).CONCLUSIONS: The data suggest that concentrations of antimicrobial peptides are correlated with severity of CF lung disease: Levels of LL-37/hCAP-18 are associated with bronchial inflammation and, therefore disease severity, whereas decreased levels of beta-defensins in advanced lung disease likely contribute to a secondary defect of the local host defense.