Yıldırım Fatih, Kutu Muhammet Emin, Çalık Yalkın, Kalkan Kübra, Akkuzu Gamze, Özgür Duygu Sevinç, Karaalioğlu Bilgin, Deniz Rabia, Güzelant Özköse Gül, İnce Burak, Bes Cemal
Department of Rheumatology, Başakşehir Çam and Sakura City Hospital, University of Health Sciences, 34480 İstanbul, Turkey.
Department of Rheumatology, Bakırköy Dr Sadi Konuk Training and Research Hospital, University of Health Sciences, 34140 İstanbul, Turkey.
Diagnostics (Basel). 2025 Apr 10;15(8):957. doi: 10.3390/diagnostics15080957.
: A limited number of previous studies have reported high rates of end-stage renal disease (ESRD) in adults with IgA vasculitis nephritis (IgAVN). Despite the high prevalence of the disease and the high rates of ESRD reported in the literature, no specific guidelines for adult patients have been established and there is no consensus on the management of the disease. This study aimed to prospectively investigate adults with IgAVN from a broad perspective. : This investigation was designed as a prospective observational study and was conducted between 01.02.2022 and 01.10.2024. A total of 49 newly diagnosed adult (>18 years) patients with IgAVN were regularly followed up. At the end of the study, the renal remission rates, factors influencing remission, treatment data, treatment-related adverse events, and disease outcomes were determined. : The median follow-up time was 22 (IQR: 11-24) months. A total of 42 patients (87%) received immunosuppressive treatment in addition to the initial glucocorticoid treatment. Azathioprine (AZA) was the preferred (41%) first steroid-sparing agent. ESRD occurred in only one patient (2%), while a total of ten patients (20%) had an unfavorable outcome. The rate of nephrotic-range proteinuria (NRP) was significantly higher in the patients who did not achieve renal remission at the end of the 12-month follow-up period (9,7% vs. 60%; = 0.02) and NRP was an independent risk factor for unfavorable outcomes [OR: 17.18; 95% CI: 1.31-224.95; = 0.03]. A total of 16% of the patients developed an infection that required hospitalization during follow-up; these patients had a higher rate of IgAVN-associated acute kidney injury (62.5% vs. 22%; = 0.02) and were significantly older (mean: 46 ± 15.3 vs. 65 ± 13.3; = 0.002). One patient died of sepsis at 4 months and another died of a myocardial infarction at 32 months. : These results suggest that adults with IgVAN do not have a high rate of ESRD if they receive effective immunosuppressive therapy. However, immunosuppressive therapy is associated with an increased risk of infection, particularly in the elderly. The presence of NRP is associated with lower long-term remission rates and has a predictive value for unfavorable outcomes.
先前数量有限的研究报告了IgA血管炎肾炎（IgAVN）成年患者中终末期肾病（ESRD）的高发病率。尽管该疾病患病率高且文献报道ESRD发病率高，但尚未制定针对成年患者的具体指南，对于该疾病的管理也未达成共识。本研究旨在从广泛的角度对成年IgAVN患者进行前瞻性调查。 ：本调查设计为前瞻性观察性研究，于2022年2月1日至2024年10月1日进行。共对49例新诊断的成年（>18岁）IgAVN患者进行定期随访。在研究结束时，确定了肾脏缓解率、影响缓解的因素、治疗数据、治疗相关不良事件和疾病结局。 ：中位随访时间为22（四分位间距：11 - 24）个月。共有42例患者（87%）在初始糖皮质激素治疗基础上接受了免疫抑制治疗。硫唑嘌呤（AZA）是首选（41%）的第一种糖皮质激素替代药物。仅1例患者（2%）发生ESRD，而共有10例患者（20%）预后不良。在12个月随访期结束时未实现肾脏缓解的患者中，肾病范围蛋白尿（NRP）发生率显著更高（9.7%对60%；P = 0.02），且NRP是预后不良的独立危险因素[比值比：17.18；95%置信区间：1.31 - 224.95；P = 0.03]。共有16%的患者在随访期间发生需要住院治疗的感染；这些患者IgAVN相关急性肾损伤发生率更高（62.5%对22%；P = 0.02），且年龄显著更大（平均：46±15.3对65±13.3；P = 0.002）。1例患者在4个月时死于败血症，另1例在32个月时死于心肌梗死。 ：这些结果表明，成年IgVAN患者若接受有效的免疫抑制治疗，ESRD发病率并不高。然而，免疫抑制治疗与感染风险增加相关，尤其是在老年人中。NRP的存在与较低的长期缓解率相关，且对不良结局具有预测价值。
