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Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis

机译：Churg-Strauss综合征或嗜酸性肉芽肿伴多血管炎

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Eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA) is a systemic small-to-medium-sized vasculitis associated with asthma and eosinophilia. Histologically EGPA presents tissue eosinophilia, necrotizing vasculitis, and granulomatous inflammation with eosinophil tissue infiltration. EGPA commonly involves the upper airway and lung parenchyma, peripheral neuropathy, cardiac disorders, and skin lesions. The anti-neutrophil cytoplasmic antibodies (ANCA) are positive in 40% of cases, especially in those patients with clinical signs of vasculitis. The pathogenesis of EGPA is multifactorial. The disease can be triggered by exposure to a variety of allergens and drugs, but a genetic background has also been described, particularly an association with HLA-DRB4. Th2 response is of special importance in the upregulation of different interleukins such as IL-4, IL-13, and IL-5. Th1 and Th17 responses are also of significance. Activated eosinophils have a prolonged survival and probably cause tissue damage by releasing eosinophil granule proteins, while their tissue recruitment can be regulated by chemokines such as eotaxin-3 and CCL17. Humoral immunity is also abnormally regulated, as demonstrated by excessive responses of IgG4 and IgE. EGPA has a good respond to glucocorticoids, although the combination of glucocorticoids and immunosuppressants (e.g., cyclophosphamide, azathioprine) is needed in most of cases. Newer treatment options include anti-IL-5 antibodies (mepolizumab), whose efficacy has been described in clinical trials, and anti-CD-20, a B cell-depleting agent (rituximab), reported in several case series.
机译：嗜酸性肉芽肿伴多血管炎（Churg-Strauss，EGPA）是一种与哮喘和嗜酸性粒细胞增多有关的全身性中小型血管炎。从组织学上讲，EGPA表现为组织嗜酸性粒细胞增多，坏死性血管炎和肉芽肿性炎症，伴有嗜酸性粒细胞组织浸润。 EGPA通常涉及上呼吸道和肺实质，周围神经病变，心脏疾病和皮肤病变。抗中性粒细胞胞浆抗体（ANCA）在40％的病例中呈阳性，尤其是在那些具有血管炎临床症状的患者中。 EGPA的发病机制是多因素的。接触多种过敏原和药物可触发该疾病，但也已描述了遗传背景，尤其是与HLA-DRB4的关联。 Th2反应在不同白介素（如IL-4，IL-13和IL-5）的上调中特别重要。 Th1和Th17反应也很重要。活化的嗜酸性粒细胞具有延长的存活期，并可能通过释放嗜酸性粒细胞颗粒蛋白而引起组织损伤，而其组织募集可以通过趋化因子如嗜酸性粒细胞趋化因子3和CCL17来调节。 IgG 4 和IgE的过度反应证明，体液免疫也受到异常调节。 EGPA对糖皮质激素的反应良好，尽管在大多数情况下需要将糖皮质激素和免疫抑制剂（例如环磷酰胺，硫唑嘌呤）结合使用。较新的治疗选择包括抗IL-5抗体（美泊利珠单抗）（其功效已在临床试验中描述）和抗CD-20（一种B细胞耗竭剂（利妥昔单抗）），已在多个案例系列中进行了报道。

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