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IgA血管炎相关性肾炎——成人发病疾病的预后

IgA vasculitis nephritis-outcomes in adult-onset disease.

作者信息

Stanway James, Brown Nina, Pervez Afeera, Van de Perre Els, Tollitt James, Marketos Nikolaos, Wong Nikki, Dhaygude Ajay, Ponnusamy Arvind, O'Riordan Ed, Venning Michael, Segelmark Mårten, Morgan Matthew, Jayne David, Hamilton Patrick, Pusey Charles D, Oni Louise, Salama Alan D

机构信息

UCL Centre for Nephrology, Royal Free Hospital, London, UK.

Department of Nephrology, Salford Royal Foundation Trust, Salford, UK.

出版信息

Rheumatology (Oxford). 2025 Feb 1;64(2):690-696. doi: 10.1093/rheumatology/keae030.

DOI:10.1093/rheumatology/keae030
PMID:38273659
Abstract

OBJECTIVES

IgA vasculitis (IgAV) in adults has been relatively under-investigated. Since outcomes are worse in other forms of vasculitis with increasing age, we investigated the outcomes of IgAV comparing younger adults (18-34), middle-aged adults (35-64) and elderly patients (≥64 years) focusing on kidney outcomes.

METHODS

We identified patients with renal biopsy-confirmed IgAV nephritis and collected data regarding clinical features and progression to end stage kidney disease (ESKD). The relationship between patient factors and ESKD was analysed by regression.

RESULTS

We identified 202 cases, 34% aged 18-34, 43% aged 35-64 and 23% elderly (>64 years). Median follow-up was 44 months. Elderly patients were more likely to present with ESKD (23.9%) compared with middle-aged (13.7%) and younger adults (2.9%) (χ2 11.6, P = 0.002). In patients with independent kidney function at biopsy, there was no difference in outcomes between age groups. Male gender, Black ethnicity, diabetes, histological evidence of chronic renal damage and estimated glomerular filtration rate < 30 ml/min were risk factors for development of ESKD. In this observational study 68.3% of patients received glucocorticoids and 56.9% additional immunosuppression.

CONCLUSION

Elderly patients with IgAV are more likely to have ESKD at presentation, but there is no difference in renal survival between age groups, among those presenting with independent renal function. Renal impairment at biopsy is an independent risk factor for subsequent development of ESKD. There is significant variability in the timing of kidney biopsy and management of these patients among specialist centres. Young adults have outcomes more in keeping with childhood IgAV.

摘要

目的

成人IgA血管炎(IgAV)相对研究较少。由于其他形式的血管炎随着年龄增长预后更差,我们比较了年轻成人(18 - 34岁)、中年成人(35 - 64岁)和老年患者(≥64岁)IgAV的预后,重点关注肾脏结局。

方法

我们确定了经肾活检确诊的IgAV肾炎患者,并收集了有关临床特征和进展至终末期肾病(ESKD)的数据。通过回归分析患者因素与ESKD之间的关系。

结果

我们确定了202例患者,其中34%为18 - 34岁,43%为35 - 64岁,23%为老年人(>64岁)。中位随访时间为44个月。与中年患者(13.7%)和年轻成人(2.9%)相比,老年患者更易出现ESKD(23.9%)(χ2 = 11.6,P = 0.002)。在活检时肾功能正常的患者中,各年龄组的结局无差异。男性、黑人种族、糖尿病、慢性肾损伤的组织学证据以及估计肾小球滤过率<30 ml/min是发生ESKD的危险因素。在这项观察性研究中,68.3%的患者接受了糖皮质激素治疗,56.9%的患者接受了额外的免疫抑制治疗。

结论

IgAV老年患者在就诊时更易发生ESKD,但在肾功能正常的患者中,各年龄组的肾脏生存率无差异。活检时的肾功能损害是随后发生ESKD的独立危险因素。各专科中心在肾活检时机和这些患者的管理方面存在显著差异。年轻成人的结局更符合儿童IgAV。

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