Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study.

Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study.
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DOI:
10.1016/j.ekir.2018.07.021
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发表时间:
2018-11
影响因子:
6
通讯作者:
CureGN Consortium
CureGN Consortium
中科院分区:
医学2区
文献类型:
--
作者:
Selewski DT;Ambruzs JM;Appel GB;Bomback AS;Matar RB;Cai Y;Cattran DC;Chishti AS;D'Agati VD;D'Alessandri-Silva CJ;Gbadegesin RA;Hogan JJ;Iragorri S;Jennette JC;Julian BA;Khalid M;Lafayette RA;Liapis H;Lugani F;Mansfield SA;Mason S;Nachman PH;Nast CC;Nester CM;Noone DG;Novak J;O'Shaughnessy MM;Reich HN;Rheault MN;Rizk DV;Saha MK;Sanghani NS;Sperati CJ;Sreedharan R;Srivastava T;Swiatecka-Urban A;Twombley K;Vasylyeva TL;Weaver DJ;Yin H;Zee J;Falk RJ;Gharavi AG;Gillespie BW;Gipson DS;Greenbaum LA;Holzman LB;Kretzler M;Robinson BM;Smoyer WE;Flessner M;Guay-Woodford LM;Kiryluk K;CureGN Consortium

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Cure肾小球肾病网络(CureGN)是一项66中心纵向观察性研究,在活检证实的微小病变疾病、局灶节段性肾小球硬化、膜性肾病或伊加肾病(IgAN)(包括伊加血管炎(IgAV))患者中开展。本研究描述了伊加队列的临床特征和治疗模式,包括IgAN与IgAV以及成人与儿童患者之间的比较。筛选前5年内诊断性肾活检的患者有资格加入CureGN。这是对入组时收集的临床和治疗数据的描述性分析。共有667名患者(506名IgAN,161名IgAV)组成IgAN/IgAV队列(382名成人,285名儿童)。在活检时,IgAV患者更年轻,(13.0岁vs. 29.6岁,P < 0.001),更常见于白色(89.7% vs. 78.9%,P = 0.003),肾小球滤过率较高(103.5 vs. 70.6 ml/min/1.73 m2,P < 0.001),血清白蛋白(3.4 vs. 3.8 g/dl,P < 0.001)低于IgAN组。成人和儿童IgAV患者在入组时或入组前接受免疫抑制治疗的可能性高于IgAN患者(79.5% vs. 54.0%,P < 0.001)。本报告强调了IgAV和IgAN之间以及儿童和成人之间的临床差异。我们根据疾病类型确定了免疫抑制治疗的差异。基线特征的描述将作为未来CureGN研究的基础。
The Cure Glomerulonephropathy Network (CureGN) is a 66-center longitudinal observational study of patients with biopsy-confirmed minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (IgAN), including IgA vasculitis (IgAV). This study describes the clinical characteristics and treatment patterns in the IgA cohort, including comparisons between IgAN versus IgAV and adult versus pediatric patients. Patients with a diagnostic kidney biopsy within 5 years of screening were eligible to join CureGN. This is a descriptive analysis of clinical and treatment data collected at the time of enrollment. A total of 667 patients (506 IgAN, 161 IgAV) constitute the IgAN/IgAV cohort (382 adults, 285 children). At biopsy, those with IgAV were younger (13.0 years vs. 29.6 years, P < 0.001), more frequently white (89.7% vs. 78.9%, P = 0.003), had a higher estimated glomerular filtration rate (103.5 vs. 70.6 ml/min per 1.73 m2, P < 0.001), and lower serum albumin (3.4 vs. 3.8 g/dl, P < 0.001) than those with IgAN. Adult and pediatric individuals with IgAV were more likely than those with IgAN to have been treated with immunosuppressive therapy at or prior to enrollment (79.5% vs. 54.0%, P < 0.001). This report highlights clinical differences between IgAV and IgAN and between children and adults with these diagnoses. We identified differences in treatment with immunosuppressive therapies by disease type. This description of baseline characteristics will serve as a foundation for future CureGN studies.
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