Rotschild M, Elias N, Berkowitz D, Pollak S, Shinawi M, Beck R, Bentur L
The Pediatric Pulmonary Unit and CF Clinic, Meyer Children's Hospital, Haifa, Israel.
Clin Exp Med. 2005 Jul;5(2):80-5. doi: 10.1007/s10238-005-0070-7.
Autoantibodies against bactericidal/permeability-increasing protein (BPI-ANCA) were found in patients with cystic fibrosis (CF). It is speculated that they represent a marker of the chronic endobronchial infection and sustained inflammatory response in CF. Our aim was to evaluate whether azithromycin (AZM), through its antiinflammatory effect, could affect the level of BPI-ANCA in CF patients. Eighteen patients with CF aged 5.5-36.3 years (median 15.1) were enrolled in a randomised, double-blind, placebo-controlled trial of AZM (250 mg twice a week to 10 patients) or placebo (8 patients) for 12 weeks. BPI-ANCA levels were recorded pre- and post-treatment and compared to a group of 18 matched healthy controls. Chi-square analysis, Kruskal-Wallis and Mann-Whitney tests were used to compare between the groups. Pre- and post-treatment values were compared using the Wilcoxon Signed-Ranked test. BPI-ANCA was found in 12 CF patients (67%) and four (22%) healthy subjects (P<0.001). The mean BPI-ANCA level was 3.94+/-6.15 U/ml (mean+/-SD) in healthy subjects and 38.11+/-42.34 U/ml in CF patients (P=0.023). The mean BPI-ANCA level was higher in patients with Pseudomonas aeruginosa compared to those without (64+/-35 U/ml and 25+/-41 U/ml respectively, P=0.032). No change in BPI-ANCA levels occurred in the AZM-treated patients [35 (0-127) U/ml (median (range) and 30 (0-120) U/ml, respectively] or in the placebo group [10 (0-66) U/ml and 13 (0-83) U/ml, respectively]. BPI-ANCA levels are significantly higher in patients with CF compared to healthy controls. BPIANCA levels are higher among patients colonised with P. aeruginosa. Twelve weeks of AZM therapy did not lower the BPI-ANCA level in patients with CF.
在囊性纤维化（CF）患者中发现了抗杀菌/通透性增加蛋白的自身抗体（BPI-ANCA）。据推测，它们代表了CF患者慢性支气管内感染和持续炎症反应的一个标志物。我们的目的是评估阿奇霉素（AZM）是否通过其抗炎作用影响CF患者的BPI-ANCA水平。18名年龄在5.5至36.3岁（中位数15.1岁）的CF患者被纳入一项随机、双盲、安慰剂对照试验，其中10名患者接受AZM（每周两次，每次250毫克）治疗，8名患者接受安慰剂治疗，为期12周。记录治疗前后的BPI-ANCA水平，并与18名匹配的健康对照者组成的小组进行比较。采用卡方分析、Kruskal-Wallis检验和Mann-Whitney检验对各组进行比较。治疗前后的值采用Wilcoxon符号秩和检验进行比较。在12名CF患者（67%）和4名（22%）健康受试者中发现了BPI-ANCA（P<0.001）。健康受试者的BPI-ANCA平均水平为3.94±6.15 U/ml（平均值±标准差），CF患者为38.11±42.34 U/ml（P=0.023）。与未感染铜绿假单胞菌的患者相比，感染该菌的患者BPI-ANCA平均水平更高（分别为64±35 U/ml和25±41 U/ml，P=0.032）。接受AZM治疗的患者[分别为35（0至127）U/ml（中位数（范围））和30（0至120）U/ml]以及安慰剂组[分别为10（0至66）U/ml和13（0至83）U/ml]的BPI-ANCA水平均未发生变化。与健康对照者相比，CF患者的BPI-ANCA水平显著更高。在感染铜绿假单胞菌的患者中，BPI-ANCA水平更高。12周的AZM治疗并未降低CF患者的BPI-ANCA水平。
