Pillebout Evangeline
Nephrology Unit, Saint-Louis Hospital, 75010 Paris, France.
INSERM 1149, Center of Research on Inflammation, 75870 Paris, France.
J Clin Med. 2021 May 25;10(11):2310. doi: 10.3390/jcm10112310.
Many authors suggested that IgA Vasculitis (IgAV) and IgA Nephropathy (IgAN) would be two clinical manifestations of the same disease; in particular, that IgAV would be the systemic form of the IgAN. A limited number of studies have included sufficient children or adults with IgAN or IgAV (with or without nephropathy) and followed long enough to conclude on differences or similarities in terms of clinical, biological or histological presentation, physiopathology, genetics or prognosis. All therapeutic trials available on IgAN excluded patients with vasculitis. IgAV and IgAN could represent different extremities of a continuous spectrum of the same disease. Due to skin rash, patients with IgAV are diagnosed precociously. Conversely, because of the absence of any clinical signs, a renal biopsy is practiced for patients with an IgAN to confirm nephropathy at any time of the evolution of the disease, which could explain the frequent chronic lesions at diagnosis. Nevertheless, the question that remains unsolved is why do patients with IgAN not have skin lesions and some patients with IgAV not have nephropathy? Larger clinical studies are needed, including both diseases, with a common histological classification, and stratified on age and genetic background to assess renal prognosis and therapeutic strategies.
许多作者认为,IgA血管炎(IgAV)和IgA肾病(IgAN)是同一种疾病的两种临床表现;特别是,IgAV是IgAN的全身形式。少数研究纳入了足够数量的患有IgAN或IgAV(有或无肾病)的儿童或成人,并进行了足够长时间的随访,以得出临床、生物学或组织学表现、病理生理学、遗传学或预后方面的差异或相似性。所有关于IgAN的现有治疗试验都排除了血管炎患者。IgAV和IgAN可能代表同一疾病连续谱的不同极端情况。由于皮疹,IgAV患者能较早被诊断出来。相反,由于没有任何临床症状,IgAN患者在疾病发展的任何阶段都需要进行肾活检以确诊肾病,这可能解释了诊断时频繁出现慢性病变的原因。然而,尚未解决的问题是,为什么IgAN患者没有皮肤病变,而一些IgAV患者没有肾病?需要开展更大规模的临床研究,纳入这两种疾病,采用共同的组织学分类,并按年龄和遗传背景进行分层,以评估肾脏预后和治疗策略。