Gan Ming Ying, Chua Freda Zhi Yun, Chang Zi Yun, Chua Yan Ting, Chan Gek Cher
Department of Medicine, National University Hospital, Singapore 119074, Singapore.
Division of Nephrology, Department of Medicine, National University Hospital, Singapore 119074, Singapore.
Life (Basel). 2024 Jul 25;14(8):930. doi: 10.3390/life14080930.
IgA vasculitis (IgAV), formerly Henoch-Schonlein purpura, is the most common systemic vasculitis in childhood. In adults, however, this condition is poorly understood, yet associated with more severe disease and poorer outcomes. This necessitates the need for early diagnosis and management.
We describe the pathophysiology, clinical manifestations, and diagnosis of IgAV in adults. Poor outcomes are often due to the high frequency of glomerulonephritis in IgAV-IgA vasculitis-associated nephritis (IgAVN). We hence also aim to summarize the latest clinical data regarding treatment strategies in IgAVN. The diagnosis and differentiation in histology between IgAVN and IgA nephropathy (IgAN) remain a challenge. Review of treatment therapies: Pathological mechanisms between IgAVN and IgAN appear to be consistent between the two, and data from IgAN are often extrapolated to IgAVN. The role of various immunosuppression therapies remains controversial, and in this review, we will discuss immunosuppression use and highlight evidence surrounding emerging and promising novel therapies in IgAVN/IgAN. Our aim for this review is to guide future treatment strategies and direct future studies.
IgA血管炎（IgAV），既往称为过敏性紫癜，是儿童期最常见的系统性血管炎。然而，在成人中，这种疾病的了解较少，但与更严重的疾病和更差的预后相关。这就需要早期诊断和管理。
我们描述了成人IgA血管炎的病理生理学、临床表现和诊断。不良预后往往归因于IgA血管炎相关肾炎（IgAVN）中肾小球肾炎的高发病率。因此，我们还旨在总结关于IgAVN治疗策略的最新临床数据。IgAVN与IgA肾病（IgAN）在组织学上的诊断和鉴别仍然是一个挑战。治疗方法综述：IgAVN和IgAN之间的病理机制似乎一致，IgAN的数据常被外推用于IgAVN。各种免疫抑制疗法的作用仍存在争议，在本综述中，我们将讨论免疫抑制疗法的使用，并强调IgAVN/IgaN中新兴和有前景的新疗法的相关证据。我们本次综述的目的是指导未来的治疗策略并指导未来的研究。
