抗BPIFA1/SPLUNC1：一种在终末期囊性纤维化患者中普遍存在的新型自身抗体。
Anti-BPIFA1/SPLUNC1: a new autoantibody prevalent in patients with endstage cystic fibrosis.
作者信息
Budding K, van de Graaf E A, Hoefnagel T, Hack C E, Otten H G
机构信息
Laboratory for Translational Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands.
Department of Respiratory Medicine, University Medical Centre Utrecht, Utrecht, The Netherlands.
出版信息
J Cyst Fibros. 2014 May;13(3):281-8. doi: 10.1016/j.jcf.2013.10.005. Epub 2013 Oct 23.
BACKGROUND
Bactericidal/permeability increasing protein fold containing family A (BPIFA) 1, is a secreted protein of the upper airways that shares structural homology with BPI and exhibits comparable antimicrobial capacities. We hypothesized that CF patients have circulating IgG or IgA anti-BPIFA1 autoantibodies, similarly as reported for BPI autoantibodies.
METHODS
We analyzed pre- and post-transplantation sera from 67 endstage lung disease patients who underwent lung transplantation (LTx) because of COPD (n=27), CF (n=25), and ILD (n=15).
RESULTS
Anti-BPIFA1 (48%) and anti-BPI (92%) were elevated in CF patients compared to healthy controls, with anti-BPIFA1 IgG isotype being most prevalent, whereas anti-BPI is of the IgA isotype. Levels of anti-BPI autoantibodies significantly declined post-LTx, whereas anti-BPIFA1 did not. No relation was found between autoantibodies against BPIFA1 and BPI.
CONCLUSION
Our results indicate that BPIFA1 is a novel target for autoantibodies in CF. The function of these autoantibodies needed to be investigated in future studies.
背景
含杀菌/通透性增加蛋白折叠家族A（BPIFA）1是上呼吸道的一种分泌蛋白，与杀菌/通透性增加蛋白（BPI）具有结构同源性，并表现出类似的抗菌能力。我们推测，与已报道的BPI自身抗体情况类似，囊性纤维化（CF）患者体内存在循环IgG或IgA抗BPIFA1自身抗体。
方法
我们分析了67例因慢性阻塞性肺疾病（COPD，n = 27）、CF（n = 25）和间质性肺疾病（ILD，n = 15）接受肺移植（LTx）的终末期肺病患者移植前后的血清。
结果
与健康对照相比，CF患者体内抗BPIFA1（48%）和抗BPI（92%）水平升高，其中抗BPIFA1 IgG同种型最为常见，而抗BPI为IgA同种型。LTx后抗BPI自身抗体水平显著下降，而抗BPIFA1水平未下降。未发现抗BPIFA1和抗BPI自身抗体之间存在关联。
结论
我们的结果表明，BPIFA1是CF患者自身抗体的一个新靶点。这些自身抗体的功能有待未来研究进一步探究。
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