Proinflammatory Cytokines and Antiskin Autoantibodies in Patients With Inherited Epidermolysis Bullosa.
Proinflammatory Cytokines and Antiskin Autoantibodies in Patients With Inherited Epidermolysis Bullosa.
复制标题
DOI：
10.1097/md.0000000000001528
复制
发表时间：
2015-10
期刊：
影响因子：
1.6
通讯作者：
Iannone F
中科院分区：
文献类型：
--
作者：
Annicchiarico G;Morgese MG;Esposito S;Lopalco G;Lattarulo M;Tampoia M;Bonamonte D;Brunetti L;Vitale A;Lapadula G;Cantarini L;Iannone F
大疱性表皮病（EB）是一种罕见的疾病，其特征是遗传性皮肤粘连缺陷，并伴有机械创伤引起的表皮-真皮连接异常破坏。我们的目的是研究一组血清样本中的细胞因子水平患有单纯性大疱性表皮病（EBS），营养不良性大疱性表皮病（DEB），和健康对照（HC），探索其潜在的相关性与抗皮肤自身抗体滴度和疾病活动。40例患者传入皮肤科病房的巴里市医院和9 HC入组，并细分为营养不良（DEB）和单纯型（EBS）。我们发现DEB（P = 0.0224）和EBS（P = 0.0465）患者的白细胞介素（IL）-1β血浆水平显著高于HC; DEB中的IL-6水平显著高于EBS患者（P = 0.0004）或HC（P = 0.0474）; DEB中的IL-2水平显著高于EBS（P = 0.0428）。 DEB患者血浆肿瘤坏死因子-β和干扰素-γ水平高于HC患者（P = 0.0448和0.0229）。 相反，肿瘤坏死因子-α在DEB中显著降低（P = 0.0034）。 IL-5与抗BP 180（r =-0.5018，P = 0.0338）、抗BP 230（r =-0.6097，P = 0.0122）和抗VII型胶原（r =-0.5166，P = 0.0405）自身抗体相关;干扰素-γ与抗BP 180（r = 0.9633，P <0.0001）、抗BP 230（r = 0.9071，P <0.0001）和抗VII型胶原（r = 0.8619，P = 0.0045）自身抗体相关。 疾病严重程度评分与IL-6（r = 0.6941，P = 0.029）和IL-12（r = 0.5503，P = 0.0272）显著相关。 目前的研究支持EB可能被认为是一种全身性炎症性疾病，而不是一种皮肤局限性疾病;临床疾病活动度评分也可以通过实验室数据（如IL-6和IL-12剂量）进行整合;针对特定细胞因子网络的生物治疗可能代表未来的发展方向。
Epidermolysis bullosa (EB) is a rare disorder characterized by inherited skin adhesion defects with abnormal disruption of the epidermal–dermal junction in response to mechanical trauma. Our aim was to investigate a set of cytokine levels in serum samples from patients suffering from epidermolysis bullosa simplex (EBS), dystrophic epidermolysis bullosa (DEB), and healthy controls (HCs), exploring their potential correlations with antiskin autoantibody titers and disease activity. Forty patients afferent to the Dermatological Ward of Bari City Hospital and 9 HCs were enrolled and subdivided according to the dystrophic (DEB) and simplex forms (EBS). We found a significant increase in interleukin (IL)-1β plasmatic levels of DEB (P = 0.0224) and EBS (P = 0.0465) patients compared to HCs; IL-6 levels were significantly higher in DEB than in EBS patients (P = 0.0004) or HCs (P = 0.0474); IL-2 levels were significantly increased in DEB compared with EBS (P = 0.0428). Plasmatic tumor necrosis factor-β and interferon-γ were higher in DEB patients than in HCs (P = 0.0448 and 0.0229). Conversely, tumor necrosis factor-α was significantly decreased in DEB (P = 0.0034). IL-5 correlated with anti-BP180 (r = −0.5018, P = 0.0338), anti-BP230 (r = −0.6097, P = 0.0122), and anticollagen VII (r = −0.5166, P = 0.0405) autoantibodies; interferon-γ correlated with anti-BP180 (r = 0.9633, P < 0.0001), anti-BP230 (r = 0.9071, P < 0.0001), and anticollagen VII (r = 0.8619, P = 0.0045) autoantibodies. Score of disease severity was significantly correlated with IL-6 (r = 0.6941, P = 0.029) and IL-12 (r = 0.5503, P = 0.0272). The present study supports that EB might be considered a systemic inflammatory disease rather than a skin-limited disorder; clinical disease activity scores could be also integrated by laboratory data such as IL-6 and IL-12 dosage; biotherapies targeting specific cytokine networks probably represent a way to go in the future.