Rosato Edoardo, Gigante Antonietta, Barbano Biagio, Gasperini Maria Ludovica, Cianci Rosario, Muscaritoli Maurizio
Department of Clinical Medicine-Scleroderma Unit, Sapienza University of Rome, Rome, Italy.
Department of Clinical Medicine-Scleroderma Unit, Sapienza University of Rome, Rome, Italy,
Kidney Blood Press Res. 2018;43(3):682-689. doi: 10.1159/000489740. Epub 2018 May 10.
BACKGROUND/AIMS: Renal involvement is common in systemic sclerosis (SSc), including asymptomatic reduction of glomerular filtration rate (GFR), increased renal resistance indices, scleroderma renal crisis (SRC) and ANCA-associated vasculitis. The aim was to evaluate type and evolution of renal involvement for a period of five years.
121 SSc patients (100 F, 21 M) with mean age of 54.9 ± 13.8, disease duration of 9 ± 6 years, of which 62 had a diffused form and 59 limited form were enrolled. All patients were screened annually for renal function by laboratory examination, ultrasound and color Doppler ultrasound of renal arteries.
Over the five-year observation period, 6 SRC (3 M, 3 F) occurred, four of which required dialysis. One patient developed ANCA-related proliferative glomerulonephritis and the other one acute tubular necrosis. The remaining 113 patients had a preserved renal function (serum creatinine 0.75 ± 0.24 mg/dl, GFR 93.8 ± 20 ml/min, 24h proteinuria 0.20 ± 0.15 g). Doppler indices of intrarenal arterial stiffness increased with progression of capillaroscopic damage and with presence of digital ulcers. A negative correlation was observed between estimated GFR and pulsatile index (p< 0,05, r=-0.198), resistive index(p< 0,01, r=0.267), S/D ratio (p< 0,01, r=-0.237).
In SSc patients, renal function was normal for 4.1 years despite the presence of increased intrarenal arterial stiffness. SRC was observed in 4.9% of SSc patients. In SSc patients, a periodic follow-up based on clinical and laboratory evaluation, colorDoppler ultrasound and, in some cases, renal biopsy is required to evaluate renal involvement.
背景/目的:肾脏受累在系统性硬化症(SSc)中很常见,包括无症状的肾小球滤过率(GFR)降低、肾阻力指数升高、硬皮病肾危象(SRC)和抗中性粒细胞胞浆抗体(ANCA)相关血管炎。目的是评估五年内肾脏受累的类型和演变情况。
纳入121例SSc患者(100例女性,21例男性),平均年龄54.9±13.8岁,病程9±6年,其中62例为弥漫型,59例为局限型。每年通过实验室检查、超声及肾动脉彩色多普勒超声对所有患者进行肾功能筛查。
在五年观察期内,发生了6例SRC(3例男性,3例女性),其中4例需要透析。1例患者发生ANCA相关的增殖性肾小球肾炎,另1例发生急性肾小管坏死。其余113例患者肾功能正常(血清肌酐0.75±0.24mg/dl,GFR 93.8±20ml/min,24小时蛋白尿0.20±0.15g)。肾内动脉僵硬度的多普勒指数随着毛细血管镜损伤的进展和指端溃疡的出现而增加。估计的GFR与搏动指数(p<0.05,r=-0.198)、阻力指数(p<0.01,r=0.267)、S/D比值(p<0.01,r=-0.237)之间存在负相关。
在SSc患者中,尽管肾内动脉僵硬度增加,但肾功能在4.1年内保持正常。4.9%的SSc患者发生了SRC。对于SSc患者,需要基于临床和实验室评估、彩色多普勒超声,以及在某些情况下进行肾活检的定期随访来评估肾脏受累情况。