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Validation of the absolute renal risk of dialysis/death in adults with IgA nephropathy secondary to Henoch-Sch?nlein purpura: a monocentric cohort study

机译:单核中心队列研究验证了成年人Henoch-Sch?nlein紫癜继发IgA肾病的透析/死亡的绝对肾脏风险:

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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-Sch?nlein 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.
机译:背景:我们较早地确定了原发性IgA肾病(IgAN)透析/死亡(D / D)的绝对肾脏风险(ARR),从而可以对最终预后进行准确的前瞻性预测。该ARR基于最初诊断时可能存在的三个主要,独立且均等的危险因素,例如高血压,定量蛋白尿?≥?1 g /天以及严重的病理性病变(通过我们的本地分类评分≥8)(范围0–20)。我们在继发性IgAN中研究了该ARR概念的有效性,以预测未来结果,并重点研究了过敏性紫癜(HSP)肾炎。方法我们的成人IgAN队列涉及1064例101例继发性IgAN,并以74例HSP(59例男性)为研究对象,初次诊断的平均年龄为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和我们的整体光学评分≥8(GOS整合了所有基本组织学病变)所赞赏的严重病理病变,其存在定义了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。主要终点是复合的,在此之前发生透析或死亡(D / D)。我们使用了经典统计数据,并使用了时变Cox回归和Kaplan-Meier生存曲线方法。结果发病后10年和20年,ARR?=?0的D / D累积率分别为0%和14%(23例患者)。对于ARR?=?1(N?=?19)为10%和23%;对于ARR≥2(N≥24),分别为27%和33%。在8例ARR?=?3(P?=?0.0007)的患者中,分别为81%和100%(在20岁之前)。诊断时(零时)的D / D事件累积率的预测值为10%(对于ARR?=?0、10%(对于ARR?=?1),33%(对于ARR?=?2)和100%)。在这个经过充分治疗的队列中,ARR?=?3(P?=?0.0003)为8.5年。结论本研究清楚地证实了新的HSP-IgAN队列中的绝对肾脏透析/死亡风险,可用于个体管理和未来的临床试验。

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