Department of Nephrology, Radboud University Medical Center, Nijmegen, the Netherlands.
Department of Nephrology, Radboud University Medical Center, Nijmegen, the Netherlands.
Am J Kidney Dis. 2017 May;69(5):637-646. doi: 10.1053/j.ajkd.2016.10.032. Epub 2017 Jan 12.
Few studies have examined the treatment and outcome of adult-onset minimal change nephrotic syndrome (MCNS). We retrospectively studied 125 patients who had MCNS with onset in either adulthood or late adolescence. Presenting characteristics, duration of initial treatment and response to treatment, relapse patterns, complications, and long-term outcome were studied.
Case series.
SETTING & PARTICIPANTS: Patients with new-onset nephrotic syndrome 16 years or older and a histologic diagnosis of MCNS in 1985 to 2011 were identified from pathology records of 10 participating centers.
Partial and complete remission, treatment resistance, relapse, complications, renal survival.
Corticosteroids were given as initial treatment in 105 (84%) patients. After 16 weeks of corticosteroid treatment, 92 (88%) of these patients had reached remission. Median time to remission was 4 (IQR, 2-7) weeks. 7 (6%) patients initially received cyclophosphamide with or without corticosteroids, and all attained remission after a median of 4 (IQR, 3-11) weeks. 13 (10%) patients reached remission without immunosuppressive treatment. One or more relapses were observed in 57 (54%) patients who received initial corticosteroid treatment. Second-line cyclophosphamide resulted in stable remission in 57% of patients with relapsing MCNS. Acute kidney injury was observed in 50 (40%) patients. Recovery of kidney function occurred almost without exception. Arterial or venous thrombosis occurred in 11 (9%) patients. At the last follow-up, 113 (90%) patients were in remission and had preserved kidney function. 3 patients with steroid-resistant MCNS progressed to end-stage renal disease, which was associated with focal segmental glomerulosclerosis lesions on repeat biopsy.
Retrospective design, variable treatment protocols.
The large majority of patients who had MCNS with onset in adulthood or late adolescence were treated with corticosteroids and reached remission, but many had relapses. Cyclophosphamide resulted in stable remission in many patients with relapses. Significant morbidity was observed due to acute kidney injury and other complications. Progression to end-stage renal disease occurred in a few patients and was explained by focal segmental glomerulosclerosis.
鲜有研究探讨成人起病的微小病变肾病综合征（MCNS）的治疗和预后。我们回顾性研究了 125 例成人或青春期后期起病的 MCNS 患者。研究了首发特征、初始治疗持续时间和治疗反应、复发模式、并发症和长期预后。
病例系列研究。
1985 年至 2011 年间，10 个参与中心的病理记录中发现了 16 岁及以上新诊断为肾病综合征且组织学诊断为 MCNS 的患者。
部分缓解和完全缓解、治疗抵抗、复发、并发症、肾脏存活率。
105 例（84%）患者接受了初始皮质激素治疗。皮质激素治疗 16 周后，92 例（88%）患者达到缓解。中位缓解时间为 4（IQR，2-7）周。7（6%）例患者最初接受环磷酰胺联合或不联合皮质激素治疗，中位时间为 4（IQR，3-11）周后均达到缓解。13（10%）例患者未接受免疫抑制治疗而达到缓解。接受初始皮质激素治疗的 57 例（54%）患者中观察到 1 次或多次复发。二线环磷酰胺治疗使 57%的复发性 MCNS 患者稳定缓解。50 例（40%）患者发生急性肾损伤，肾功能几乎无一例外地恢复。11（9%）例患者发生动静脉血栓形成。最后一次随访时，113 例（90%）患者缓解且肾功能正常。3 例类固醇耐药性 MCNS 患者进展至终末期肾病，这与重复肾活检时出现局灶节段性肾小球硬化病变有关。
回顾性设计，治疗方案多变。
大多数成人或青春期后期起病的 MCNS 患者接受皮质激素治疗并达到缓解，但许多患者复发。环磷酰胺治疗使许多复发患者稳定缓解。急性肾损伤和其他并发症导致大量患者出现严重并发症。少数患者进展至终末期肾病，这与局灶节段性肾小球硬化有关。
