J Biol Regul Homeost Agents. 2012 Jan-Mar;26(1):135-8.
Idiopathic membranous glomerulonephritis is a frequent cause of nephrotic syndrome and may have a variable course, from spontaneous remission to progression on renal failure. The therapy is based on alternating steroids and chlorambucil or cyclophosphamide (Ponticelli protocol) for six months. In absence of complete or partial remission after protocol, cyclosporine, adrenocorticotropic hormone, mycophenolate mofetil, rituximab can be used for potential therapy. We report here the case of a woman with idiopathic membranous glomerulonephritis unresponsive to the Ponticelli regimen and treated with adrenocorticotropic hormone in association with azathioprine, showing a dramatic decrease of proteinuria and beneficial effects on lipid profile. After 36 months, no relapse of disease has occurred. Although larger cohorts of patients are needed to evaluate the long-term effects, adrenocorticotropic hormone plus azathioprine in association could be a possible therapeutic option for unresponsive idiopathic membranous glomerulonephritis.
特发性膜性肾小球肾炎是肾病综合征的常见病因,其病程可能多变,从自发性缓解到肾功能衰竭进展。治疗基于皮质类固醇和苯丁酸氮芥或环磷酰胺(Ponticelli 方案)交替治疗六个月。如果方案后没有完全或部分缓解,可以使用环孢素、促肾上腺皮质激素、霉酚酸酯、利妥昔单抗进行潜在治疗。我们在此报告一例特发性膜性肾小球肾炎患者,对 Ponticelli 方案无反应,并用促肾上腺皮质激素联合硫唑嘌呤治疗,蛋白尿显著减少,血脂谱改善。36 个月后,疾病未复发。尽管需要更大的患者队列来评估长期效果,但促肾上腺皮质激素加硫唑嘌呤联合治疗可能是治疗特发性膜性肾小球肾炎无反应的一种可能的治疗选择。