Validation of the absolute renal risk of dialysis/death in adults with IgA nephropathy secondary to Henoch-Schonlein purpura: a monocentric cohort study
Validation of the absolute renal risk of dialysis/death in adults with IgA nephropathy secondary to Henoch-Schonlein purpura: a monocentric cohort study
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DOI：
10.1186/1471-2369-14-169
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发表时间：
2013-08-01
期刊：
影响因子：
2.3
通讯作者：
Berthoux, Francois
中科院分区：
文献类型：
--
作者：
Mohey, Hesham;Laurent, Blandine;Berthoux, Francois
背景：我们较早建立了原发性 IgA 肾病 (IgAN) 透析/死亡 (D/D) 的绝对肾脏风险 (ARR)，这使得能够准确地前瞻性预测最终预后。该 ARR 是基于初始诊断时可能存在的三个主要、独立且等价的危险因素，例如高血压、定量蛋白尿 >= 1 g/天，以及我们的本地分类评分 >= 8（范围 0-20）评估的严重病理病变。我们研究了继发性 IgAN 中 ARR 概念的有效性，以预测未来的结果，并重点关注过敏性紫癜 (HSP) 肾炎。方法：我们的成人 IgAN 队列涉及 1064 名患有 101 例继发性 IgAN 的患者，重点关注 74 名 HSP（59 名男性），初次诊断时的平均年龄为 38.6 岁，平均随访时间为 11.8 年。在活检证实的诊断中研究了三个主要危险因素：高血压、蛋白尿 >= 1 g/d 以及我们的总体光学评分 >= 8 所识别的严重病理病变（GOS 整合了所有基本组织学病变），它们的存在定义了 ARR 评分：0 表示不存在，3 表示全部存在，1 或 2 表示存在任何 1 或 2 个危险因素。主要终点是与之前发生透析或死亡的复合终点（D/D）。我们使用经典统计、时间依赖性Cox回归和Kaplan-Meier生存曲线方法。结果：对于ARR=0（23名患者），发病后10年和20年的D/D累积率分别为0%和14%； ARR = 1 时为 10% 和 23%（N = 19）； ARR = 2 (N = 24) 时为 27% 和 33%； 8 名 ARR = 3 的患者中分别为 81% 和 100%（20 岁前）（P = 0.0007）。在这个经过充分治疗的队列中，诊断时（零时间）D/D 事件 10 年累积率的预测为：ARR = 0 时为 0%，ARR = 1 时为 10%，ARR = 2 时为 33%，ARR = 3 时为 33%（P = 0.0003）。结论：这项研究在新的队列中明确验证了透析/死亡的绝对肾脏风险概念。 HSP-IgAN 可用于个体管理和未来的临床试验。
Background: We established earlier the absolute renal risk (ARR) of dialysis/death (D/D) in primary IgA nephropathy (IgAN) which permitted accurate prospective prediction of final prognosis. This ARR was based on the potential presence at initial diagnosis of three major, independent, and equipotent risk factors such as hypertension, quantitative proteinuria >= 1 g per day, and severe pathological lesions appreciated by our local classification scoring >= 8 (range 0-20). We studied the validity of this ARR concept in secondary IgAN to predict future outcome and focused on Henoch-Schonlein purpura (HSP) nephritis.Methods: Our cohort of adults IgAN concerned 1064 patients with 101 secondary IgAN and was focused on 74 HSP (59 men) with a mean age of 38.6 at initial diagnosis and a mean follow-up of 11.8 years. Three major risk factors: hypertension, proteinuria >= 1 g/d, and severe pathological lesions appreciated by our global optical score >= 8 (GOS integrated all elementary histological lesions), were studied at biopsy-proven diagnosis and their presence defined the ARR scoring: 0 for none present, 3 for all present, 1 or 2 for the presence of any 1 or 2 risk factors. The primary end-point was composite with occurrence of dialysis or death before (D/D). We used classical statistics and both time-dependent Cox regression and Kaplan-Meier survival curve methods.Results: The cumulative rate of D/D at 10 and 20 years post-onset was respectively 0 and 14% for ARR = 0 (23 patients); 10 and 23% for ARR = 1 (N = 19); 27 and 33% for ARR = 2 (N = 24); and 81 and 100% (before 20 y) in the 8 patients with ARR = 3 (P = 0.0007). Prediction at time of diagnosis (time zero) of 10y cumulative rate of D/D event was 0% for ARR = 0, 10% for ARR = 1, 33% for ARR = 2, and 100% by 8.5y for ARR = 3 (P = 0.0003) in this adequately treated cohort.Conclusion: This study clearly validates the Absolute Renal Risk of Dialysis/Death concept in a new cohort of HSP-IgAN with utility to individual management and in future clinical trials.