Hočevar Alojzija, Ostrovršnik Jaka, Jurčić Vesna, Tomšič Matija, Rotar Žiga
Department of Rheumatology, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Front Med (Lausanne). 2023 Jul 17;10:1210307. doi: 10.3389/fmed.2023.1210307. eCollection 2023.
Follow-up data on IgA vasculitis (IgAV) in adults are scarce. We aimed to investigate the outcome of adult IgAV in a well-defined cohort.
Data from histologically proven patients diagnosed between January 2010 and July 2022 with at least a 3-month follow-up were analyzed. The frequency and type of relapses and information on kidney function were extracted. Risk factors for IgAV relapse and decline in renal function were studied using the Cox hazards regression analysis. Mortality in IgAV was assessed using the Kaplan-Meier analysis and the standardized mortality ratio (SMR).
In total, 265 patients were followed for a median of 24 months. At baseline, 38.9, 29.8, and 44.5% had articular, gastrointestinal, and renal involvement, respectively. Initially, 189 (71.3%) patients received systemic glucocorticoids, and 32 (12.1%) patients received an additional immunomodulator. During follow-up, 42 (15.8%) patients relapsed. Relapses were more common in younger patients (HR 1.03 [95%CI 1.01-1.05]) and those without baseline glucocorticoid treatment (HR 3.70 [95%CI 2.0-6.67]). Furthermore, 74 (27.9%) patients had persistent abnormal urinalysis and a substantial (≥20%) decline in glomerular filtration rate (eGFR) was recorded in 41 (15.5%) patients. The factors associated with persistent abnormal urinalysis were an absence of IgAV joint involvement and baseline immunomodulatory treatment. Pre-existent chronic kidney disease and heart failure were associated with eGFR decline. The overall SMR was 1.4 (95%CI 1.14-1.71) compared to the Slovenian general population.
IgAV relapses occurred in 15% of patients, with younger patients with symptomatically managed IgAV experiencing it more frequently. Heart failure emerged as a predictor of persistent abnormal urinalysis and a decline in eGFR. Adults with IgAV had increased mortality compared to the general population.
成人IgA血管炎（IgAV）的随访数据稀缺。我们旨在调查一个明确队列中成人IgAV的结局。
分析2010年1月至2022年7月间经组织学证实、随访至少3个月的患者数据。提取复发频率和类型以及肾功能信息。使用Cox风险回归分析研究IgAV复发和肾功能下降的危险因素。采用Kaplan-Meier分析和标准化死亡率（SMR）评估IgAV的死亡率。
总共对265例患者进行了中位24个月的随访。基线时，分别有38.9%、29.8%和44.5%的患者有关节、胃肠道和肾脏受累。最初，189例（71.3%）患者接受了全身糖皮质激素治疗，32例（12.1%）患者接受了额外的免疫调节剂治疗。随访期间，42例（15.8%）患者复发。复发在年轻患者中更常见（HR 1.03 [95%CI 1.01 - 1.05]）以及未接受基线糖皮质激素治疗的患者中更常见（HR 3.70 [95%CI 2.0 - 6.67]）。此外，74例（27.9%）患者尿常规持续异常，41例（15.5%）患者肾小球滤过率（eGFR）大幅下降（≥20%）。与尿常规持续异常相关的因素是无IgAV关节受累和基线免疫调节治疗。既往慢性肾脏病和心力衰竭与eGFR下降相关。与斯洛文尼亚普通人群相比，总体SMR为1.4（95%CI 1.14 - 1.71）。
15%的患者发生IgAV复发，症状性治疗的年轻IgAV患者复发更频繁。心力衰竭是尿常规持续异常和eGFR下降的预测因素。与普通人群相比，IgAV成人患者死亡率增加。
