Assistance Publique - Hôpitaux de Paris, Soins Intensifs Néphrologiques et Rein Aigu, APHP Sorbonne Université, Hôpital Tenon, Paris, France.
French Intensive Care Renal Network, Marseille France.
Nephrol Dial Transplant. 2023 Jul 31;38(8):1848-1856. doi: 10.1093/ndt/gfac324.
Nephrosclerosis is one of the histopathological consequences of severe or malignant hypertension (MH), some of the pathophysiology of which has been extrapolated from essential polygenetic arterial hypertension. Despite our recent description of unsuspected ciliopathies with MH, causes of MH in young patients with severe renal impairment are poorly understood.
To refine and better describe the MH phenotype, we studied clinical and prognostic factors in young patients receiving a kidney biopsy following their first episode of MH. Patients were identified retrospectively and prospectively from eight centres over a 35-year period (1985-2020). Keywords were used to retrospectively enrol patients irrespective of lesions found on renal biopsy.
A total of 114 patients were included, 77 (67%) of whom were men, average age 34 years, 35% Caucasian and 34% African origin. An isolated clinical diagnosis of severe nephrosclerosis was suggested in only 52% of cases, with 24% primary glomerulopathies. Only 7% of patients had normal renal function at diagnosis, 25% required emergency dialysis and 21% were eventually transplanted. Mortality was 1% at the last follow-up. Independent prognostic factors significantly associated with renal prognosis (6-month dialysis) and predictive of end-stage renal disease were serum creatinine on admission {odds ratio [OR] 1.56 [95% confidence interval (CI) 1.34-1.96], P < .001} and renal fibrosis >30% [OR 10.70 (95% CI 1.53-112.03), P = .03]. Astonishingly, the presence of any thrombotic microangiopathy lesion on renal biopsy was an independent, protective factor [OR 0.14 (95% CI 0.02-0.60), P = .01]. The histopathological hallmark of nephrosclerosis was found alone in only 52% of study patients, regardless of ethnicity.
This suggests that kidney biopsy might be beneficial in young patients with MH.
肾硬化是严重或恶性高血压（MH）的组织病理学后果之一，其部分病理生理学已从原发性多基因动脉高血压推断而来。尽管我们最近描述了伴有 MH 的未被怀疑的纤毛病，但年轻严重肾功能损害患者发生 MH 的原因仍知之甚少。
为了完善和更好地描述 MH 表型，我们研究了首次发生 MH 后接受肾活检的年轻患者的临床和预后因素。在 35 年期间（1985-2020 年），我们从 8 个中心回顾性和前瞻性地确定了患者。使用关键词回顾性招募了无论肾活检是否发现病变的患者。
共纳入 114 例患者，其中 77 例（67%）为男性，平均年龄 34 岁，35%为白种人，34%为非洲裔。仅有 52%的病例提示孤立的临床诊断为严重肾硬化，24%为原发性肾小球病。诊断时仅有 7%的患者肾功能正常，25%需要紧急透析，21%最终接受了移植。末次随访时死亡率为 1%。与肾功能预后（6 个月透析）相关的独立预后因素，并可预测终末期肾病的是入院时的血清肌酐{比值比（OR）1.56[95%置信区间（CI）1.34-1.96]，P<0.001}和肾纤维化>30%[OR 10.70（95% CI 1.53-112.03），P=0.03]。令人惊讶的是，肾活检中存在任何血栓性微血管病病变是一个独立的保护因素[OR 0.14（95% CI 0.02-0.60），P=0.01]。研究患者中，肾硬化的组织病理学标志单独出现的比例仅为 52%，无论其种族如何。
这表明肾活检可能对年轻的 MH 患者有益。
