Stangou Maria, Papasotiriou Marios, Xydakis Dimitrios, Oikonomaki Theodora, Marinaki Smaragdi, Zerbala Synodi, Stylianou Constantinos, Kalliakmani Pantelitsa, Andrikos Aimilios, Papadaki Antonia, Balafa Olga, Golfinopoulos Spyridon, Visvardis Georgios, Moustakas Georgios, Papachristou Evangelos, Kouloukourgiotou Theodora, Kapsia Eleni, Panagiotou Angeliki, Koulousios Constantinos, Kavlakoudis Christos, Georgopoulou Maria, Panagoutsos Stylianos, Vlahakos Demetrios V, Apostolou Theophanis, Stefanidis Ioannis, Siamopoulos Kostas, Tzanakis Ioannis, Papadogiannakis Apostolos, Daphnis Eugene, Iatrou Christos, Boletis John N, Papagianni Aikaterini, Goumenos Dimitrios S
Department of Nephrology, Hippokratio General Hospital, Aristotle University, Thessaloniki, Greece.
Department of Nephrology, University Hospital of Patras, Patras, Greece.
Clin Kidney J. 2018 Feb;11(1):38-45. doi: 10.1093/ckj/sfx076. Epub 2017 Jul 31.
Natural history, predisposing factors to an unfavourable outcome and the effect of various therapeutic regimens were evaluated in a cohort of 457 patients with immunoglobulin A nephropathy (IgAN) and follow-up of at least 12 months.
Patients with normal renal function and proteinuria <1 g/24 h as well as those with serum creatinine (SCr) >2.5 mg/dL and/or severe glomerulosclerosis received no treatment. Patients with normal or impaired renal function and proteinuria >1 g/24 h for >6 months received daily oral prednisolone or a 3-day course of intravenous (IV) methylprednisolone followed by oral prednisolone every other day or a combination of prednisolone and azathioprine. The clinical outcome was estimated using the primary endpoints of end-stage renal disease and/or doubling of baseline SCr.
The overall 10-year renal survival was 90.8%, while end-stage renal disease and doubling of baseline SCr developed in 9.2% and 14.7% of patients, respectively. Risk factors related to the primary endpoints were elevated baseline SCr, arterial hypertension, persistent proteinuria >0.5 g/24 h and severity of tubulointerstial fibrosis. There was no difference in the clinical outcome of patients treated by the two regimens of corticosteroids; nevertheless, remission of proteinuria was more frequent in patients who received IV methylprednisolone (P = 0.000). The combination of prednisolone with azathioprine was not superior to IV methylprednisolone followed by oral prednisolone. Side effects related to immunossuppressive drugs were observed in 12.8% of patients.
The clinical outcome of patients with IgAN was related to the severity of clinical and histological involvement. The addition of azathioprine to a corticosteroid-based regimen for IgAN does not improve renal outcome.
在一个457例免疫球蛋白A肾病(IgAN)患者队列中评估其自然病史、不良预后的诱发因素以及各种治疗方案的效果,随访时间至少为12个月。
肾功能正常且蛋白尿<1g/24小时的患者以及血清肌酐(SCr)>2.5mg/dL和/或严重肾小球硬化的患者未接受治疗。肾功能正常或受损且蛋白尿>1g/24小时持续超过6个月的患者接受每日口服泼尼松龙或3天疗程的静脉注射甲泼尼龙,随后隔日口服泼尼松龙,或泼尼松龙与硫唑嘌呤联合使用。使用终末期肾病和/或基线SCr翻倍的主要终点来评估临床结局。
总体10年肾脏生存率为90.8%,而终末期肾病和基线SCr翻倍分别发生在9.2%和14.7%的患者中。与主要终点相关的危险因素包括基线SCr升高、动脉高血压、持续性蛋白尿>0.5g/24小时以及肾小管间质纤维化的严重程度。两种糖皮质激素治疗方案的患者临床结局无差异;然而,接受静脉注射甲泼尼龙的患者蛋白尿缓解更为频繁(P = 0.000)。泼尼松龙与硫唑嘌呤联合使用并不优于静脉注射甲泼尼龙后口服泼尼松龙。12.8%的患者观察到与免疫抑制药物相关的副作用。
IgAN患者的临床结局与临床和组织学受累的严重程度有关。在基于糖皮质激素的IgAN治疗方案中添加硫唑嘌呤并不能改善肾脏结局。