Salemi M, Mandalà V, Muggeo V, Misiano G, Milano S, Colonna-Romano G, Arcoleo F, Cillari E
Patologia Clinica Presidio Ospedaliero V. Cervello, Ospedali Riuniti Villa Sofia-Cervello, Palermo, Italy.
Dipartimento di Scienze Statistiche e Matematiche "Vianelli", Università di Palermo, Palermo, Italy.
Clin Exp Med. 2016 May;16(2):213-8. doi: 10.1007/s10238-015-0340-y. Epub 2015 Mar 15.
Hereditary angioedema (HAE) is a rare autosomal dominant disorder, due to C1-inhibitor deficiency, which causes episodic swellings of subcutaneous tissues, bowel walls and upper airways which are disabling and potentially life-threatening. We evaluated n = 17 patients with confirmed HAE diagnosis in basal and crisis state and n = 19 healthy subjects. The samples were tested for IL-17, FGFb, G-CSF and GM-CSF, using Bio-plex kit. Data analysis was performed via nonparametric Spearman's correlations and two sets of linear mixed models. When comparing HAE subjects during basal and crisis states, we found out significantly (i.e., p value <0.05) higher values in crisis states rather than in basal states for the three growth factors and cytokine IL-17. When comparing healthy subjects versus HAE patients at basal state, we found out significantly higher values in HAE subjects only for GM-CSF, FGFb and IL-17, but not for G-CSF. In HAE patients, there is a connection between IL-17 and growth factors. The low-grade inflammation in absence of attacks is demonstrated by constant higher amount of IL-17, FGFb and GM-CSF with respect to healthy patients. This could indicate that in this disease there is a level of activation that maintains the system in a "tick-over state," that can be activate by several stimuli that are able to induce a increase in inflammatory mediators during the acute attack.
遗传性血管性水肿(HAE)是一种罕见的常染色体显性疾病,由于C1抑制剂缺乏,导致皮下组织、肠壁和上呼吸道的发作性肿胀,这些肿胀会使人致残并可能危及生命。我们评估了17例确诊为HAE的患者,分别处于基础状态和发作期,以及19名健康受试者。使用Bio-plex试剂盒对样本进行白细胞介素-17(IL-17)、碱性成纤维细胞生长因子(FGFb)、粒细胞集落刺激因子(G-CSF)和粒细胞-巨噬细胞集落刺激因子(GM-CSF)检测。数据分析通过非参数Spearman相关性分析和两组线性混合模型进行。在比较HAE患者的基础状态和发作期时,我们发现三种生长因子和细胞因子IL-17在发作期的值显著高于基础状态(即p值<0.05)。在比较基础状态下的健康受试者与HAE患者时,我们发现仅GM-CSF、FGFb和IL-17在HAE受试者中的值显著高于健康受试者,而G-CSF并非如此。在HAE患者中,IL-17与生长因子之间存在联系。相对于健康患者,IL-17、FGFb和GM-CSF的持续较高水平表明在无发作时存在低度炎症。这可能表明在这种疾病中存在一定程度的激活,使系统维持在“运转状态”,这种激活可由多种刺激引发,这些刺激能够在急性发作期间诱导炎症介质增加。