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[成人IgA血管炎(过敏性紫癜)]

[Adult IgA vasculitis (Henoch-Schönlein purpura)].

作者信息

Pillebout Évangéline

机构信息

Service de néphrologie et de transplantation, hôpital Saint-Louis, 1, avenue Claude-Vellefaux, 75010 Paris, France; Inserm U1149, CRI, faculté de médecine Xavier-Bichat, 16, rue Henri-Huchard, 75018 Paris, France.

出版信息

Nephrol Ther. 2019 Apr;15 Suppl 1:S13-S20. doi: 10.1016/j.nephro.2019.02.001.

Abstract

IgA vasculitis is a systemic vasculitis affecting small vessels. IgA vasculitis usually affect children whereas it is rare in adults (150 to 200 for 1) in which the disease is often more serious with more frequent and severe nephritis. Prevalence of adult IgA vasculitis is unknown and its annual incidence is 1 in 1 million. The dominant clinical features include cutaneous purpura, arthritis and gastrointestinal symptoms. Sometimes nephritis can add, typically as glomerulonephritis with IgA mesangial deposits. Pulmonary, cardiac, genital and neurological symptoms have also been observed. Although the cause is unknown, it is clear that IgA plays a pivotal role in the immunopathogenesis of IgA vasculitis. Only symptomatic treatment is advised in case of self-limited disease. Treatment of severe IgA vasculitis, nephritis or gastrointestinal manifestations, is not established but some studies, which need to be confirmed, reported the benefit of corticosteroids combined with immunosuppressive drugs. Short-term outcome depends of the severity of the gastro-intestinal manifestations. The long-term prognosis is heavily dependent on the presence and severity of nephritis. Studies with prolonged follow-up show up to one third of adult patients reaching end stage renal failure, as for IgA nephropathy. Some authors even suggest that IgA nephropathy and IgA vasculitis would be the same disease.

摘要

IgA 血管炎是一种累及小血管的系统性血管炎。IgA 血管炎通常影响儿童,而在成人中较为罕见(比例为 150 至 200 比 1),在成人中该疾病往往更严重,肾炎更频繁且更严重。成人 IgA 血管炎的患病率未知,其年发病率为百万分之一。主要临床特征包括皮肤紫癜、关节炎和胃肠道症状。有时会出现肾炎,典型表现为伴有 IgA 系膜沉积的肾小球肾炎。还观察到肺部、心脏、生殖器和神经系统症状。虽然病因不明,但很明显 IgA 在 IgA 血管炎的免疫发病机制中起关键作用。对于自限性疾病,建议仅进行对症治疗。严重 IgA 血管炎、肾炎或胃肠道表现的治疗方法尚未确定,但一些研究(有待证实)报道了糖皮质激素联合免疫抑制药物的益处。短期预后取决于胃肠道表现的严重程度。长期预后很大程度上取决于肾炎的存在和严重程度。长期随访研究表明,多达三分之一的成年患者会发展为终末期肾衰竭,这与 IgA 肾病情况相同。一些作者甚至认为 IgA 肾病和 IgA 血管炎是同一种疾病。

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