Schmitz Veronica, Prata Rhana Berto da Silva, Barbosa Mayara Garcia de Mattos, Mendes Mayara Abud, Brandão Sheila Santos, Amadeu Thaís Porto, Rodrigues Luciana Silva, Ferreira Helen, Costa Fabrício da Mota Ramalho, Dos Santos Jessica Brandão, Pacheco Fabiana Dos Santos, Machado Alice de Miranda, Nery José Augusto da Costa, Hacker Mariana de Andrea, Sales Anna Maria, Pinheiro Roberta Olmo, Sarno Euzenir Nunes
Instituto Oswaldo Cruz, Fundação Oswaldo Cruz, Rio de Janeiro, Brazil.
Faculdade de Ciências Médicas, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
PLoS Negl Trop Dis. 2016 Aug 24;10(8):e0004955. doi: 10.1371/journal.pntd.0004955. eCollection 2016 Aug.
Erythema Nodosum Leprosum (ENL) is an immune reaction in leprosy that aggravates the patient´s clinical condition. ENL presents systemic symptoms of an acute infectious syndrome with high leukocytosis and intense malaise clinically similar to sepsis. The treatment of ENL patients requires immunosuppression and thus needs to be early and efficient to prevent both disabilities and permanent nerve damage. Some patients experience multiple episodes of ENL and prolonged use of immunosuppressive drugs may lead to serious adverse effects. Thalidomide treatment is extremely effective at ameliorating ENL symptoms. Several mechanisms have been proposed to explain the efficacy of thalidomide in ENL, including the inhibition of TNF production. Given its teratogenicity, thalidomide is prohibitive for women of childbearing age. A rational search for molecular targets during ENL episodes is essential to better understand the disease mechanisms involved, which may also lead to the discovery of new drugs and diagnostic tests. Previous studies have demonstrated that IFN-γ and GM-CSF, involved in the induction of CD64 expression, increase during ENL. The aim of the present study was to investigate CD64 expression during ENL and whether thalidomide treatment modulated its expression. Leprosy patients were allocated to one of five groups: (1) Lepromatous leprosy, (2) Borderline leprosy, (3) Reversal reaction, (4) ENL, and (5) ENL 7 days after thalidomide treatment. The present study demonstrated that CD64 mRNA and protein were expressed in ENL lesions and that thalidomide treatment reduced CD64 expression and neutrophil infiltrates-a hallmark of ENL. We also showed that ENL blood neutrophils exclusively expressed CD64 on the cell surface and that thalidomide diminished overall expression. Patient classification based on clinical symptoms found that severe ENL presented high levels of neutrophil CD64. Collectively, these data revealed that ENL neutrophils express CD64, presumably contributing to the immunopathogenesis of the disease.
结节性红斑型麻风(ENL)是麻风病中的一种免疫反应,会加重患者的临床病情。ENL呈现出急性感染综合征的全身症状,伴有白细胞增多和强烈不适,临床上类似于败血症。ENL患者的治疗需要免疫抑制,因此需要早期且有效,以预防残疾和永久性神经损伤。一些患者会经历多次ENL发作,长期使用免疫抑制药物可能会导致严重的不良反应。沙利度胺治疗在改善ENL症状方面极其有效。已经提出了几种机制来解释沙利度胺在ENL中的疗效,包括抑制肿瘤坏死因子(TNF)的产生。鉴于其致畸性,沙利度胺对育龄妇女禁用。在ENL发作期间合理寻找分子靶点对于更好地理解所涉及的疾病机制至关重要,这也可能导致发现新药和诊断测试。先前的研究表明,参与诱导CD64表达的干扰素-γ(IFN-γ)和粒细胞-巨噬细胞集落刺激因子(GM-CSF)在ENL期间会增加。本研究的目的是调查ENL期间CD64的表达以及沙利度胺治疗是否会调节其表达。麻风病患者被分为五组之一:(1)瘤型麻风,(2)界线类麻风,(3)反应性麻风,(4)ENL,以及(5)沙利度胺治疗7天后的ENL。本研究表明,CD64 mRNA和蛋白在ENL病变中表达,并且沙利度胺治疗降低了CD64表达和中性粒细胞浸润——这是ENL的一个标志。我们还表明,ENL血液中的中性粒细胞仅在细胞表面表达CD64并且沙利度胺减少了总体表达。基于临床症状的患者分类发现,严重ENL患者的中性粒细胞CD64水平较高。总体而言,这些数据表明ENL中性粒细胞表达CD64,可能促成了该疾病的免疫发病机制。