嗜酸性肉芽肿性多血管炎(EGPA)是一种坏死性血管炎,通常会影响中小型血管。它的特点是存在富含嗜酸性粒细胞的组织浸润,随着肉芽肿性病变的形成。约40%的病例抗中性粒细胞胞浆抗体(ANCA)阳性,主要的核周染色,和抗髓过氧化物酶(抗MPO)特异性在约65%的病例中。EGPA的典型表现包括晚发性哮喘,鼻窦相关症状,周围神经病变,在外周血中观察到显著的嗜酸性粒细胞增多。与肉芽肿性多血管炎和显微镜下多血管炎相反,EGPA的肾脏受累频率较低(约25%),并且研究很少。在ANCA阳性的EGPA患者中,坏死性免疫新月体性肾小球肾炎是最常见的肾脏表现。虽然很少,也可以观察到其他形式的肾脏受累,如嗜酸性粒细胞性间质性肾炎,系膜肾小球肾炎,膜性肾病,或局灶性硬化症。尚未定义EGPA肾脏受累的标准化治疗方法,然而,生存率和肾脏结局通常优于其他ANCA相关血管炎.尽管如此,肾脏疾病是EGPA患者的不良预后因素.需要更大的研究来更好地描述肾脏受累,特别是对于与新月体肾小球肾炎不同的模式,并支持发展自愿的治疗方法。在这篇文章中,除了个人资料,我们将回顾基于ANCA的患者临床表型的最新发现,遗传学和生物药物对疾病管理的影响。
Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis, which typically affects small-to medium-sized blood vessels. It is characterized by the presence of tissue infiltrates rich in eosinophils, along with the formation of granulomatous lesions. About 40% of cases have positive anti-neutrophil cytoplasm antibodies (ANCA), with predominant perinuclear staining, and anti-myeloperoxidase (anti-MPO) specificity in about 65% of cases. Typical manifestations of EGPA include the late onset of asthma, nasal and sinus-related symptoms, peripheral neuropathy, and significant eosinophilia observed in the peripheral blood. In contrast to granulomatosis with polyangiitis and microscopic polyangiitis, renal involvement in EGPA is less frequent (about 25%) and poorly studied. Necrotizing pauci-immune crescentic glomerulonephritis is the most common renal presentation in patients with ANCA-positive EGPA. Although rarely, other forms of renal involvement may also be observed, such as eosinophilic interstitial nephritis, mesangial glomerulonephritis, membranous nephropathy, or focal sclerosis. A standardized treatment for EGPA with renal involvement has not been defined, however the survival and the renal outcomes are usually better than in the other ANCA-associated vasculitides. Nonetheless, kidney disease is an adverse prognostic factor for EGPA patients. Larger studies are required to better describe the renal involvement, in particular for patterns different from crescentic glomerulonephritis, and to favor the development of a consensual therapeutic approach. In this article, in addition to personal data, we will review recent findings on patient clinical phenotypes based on ANCA, genetics and the impact of biological drugs on disease management.