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Clinical Trials in ANCA Associated Vasculitis

机译:ANCA相关性血管炎的临床试验

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Wegener’s granulomatosis (WG) was described in 1936 [1], Churg-Strauss syndrome (CSS) in 1951 [2], and microscopic npolyangiitis (MPA) was formally recognized in 1994 [3]. These three conditions have been linked under the umbrella term of nANCA associated vasculitis (AAV) due to their association with antibodies directed against antigens in the neutrophil ncytoplasm (ANCA). All three conditions produce necrotising vasculitis of medium and small vessels with differing npredilections to various organs. Although these conditions have been recognized since the 1930’s, the first clinical trial was not npublished till 1983. Fauci et al. shared their experience of using open label, uncontrolled oral cyclophosphamide and nglucocorticoid combination therapy in patients with WG [4]. That paper described 85 patients recruited in one centre over 21 nyears. 27 years later, two randomised clinical trials were published in the same week [5-6]. Between the two, the papers ndescribed 245 patients with WG or MPA recruited over 4 years and followed up for predefined durations in 17 centres across nthe world. Compared to the first 60 years of WG, our understanding and thus our methodology of investigating potential ntherapies for these conditions has changed dramatically. In the last decade there have been 12 randomised controlled trials in npatients with AAV (Table 1).
机译:Wegener的肉芽肿病(WG)于1936年[1]被描述,Churg-Strauss综合征(CSS)于1951年被描述[2],微观的多发性血管炎(MPA)于1994年被正式认可[3]。这三种情况在nANCA相关性血管炎(AAV)的统称下已被关联,因为它们与针对中性粒细胞胞质(ANCA)抗原的抗体相关。所有这三种情况均会产生中,小血管的坏死性血管炎,对各个器官的影响不同。尽管自1930年代以来就已认识到这些情况,但直到1983年才进行了首次临床试验。分享了他们在WG患者中使用开放标签,不受控制的口服环磷酰胺和n糖皮质激素联合治疗的经验[4]。该论文描述了21年内在一个中心招募的85名患者。 27年后,同一周发表了两项随机临床试验[5-6]。在这两者之间,论文描述了245名WG或MPA患者,这些患者在4年内被招募,并在全球17个中心进行了预定的随访。与WG的前60年相比,我们对这些疾病潜在治疗方法的研究以及方法的研究发生了巨大变化。在过去的十年中,有12项针对AAV的n病人的随机对照试验(表1)。

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  • 来源
    《Current Immunology Reviews》 |2011年第4期|p.385-387|共3页
  • 作者

    Chetan Mukhtyar;

  • 作者单位

    Norfolk and Norwich University Hospital University of East Anglia Colney Lane Norwich NR4 7PD UK;

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  • 正文语种 eng
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