Das U, Dakshinamurty K V, Prasad N
Department of Nephrology, Nizam's Institute of Medical Sciences, Hyderabad, India.
Indian J Nephrol. 2009 Apr;19(2):48-52. doi: 10.4103/0971-4065.53321.
Various studies have demonstrated that treatment with methyl prednisolone and chlorambucil could increase the chance of remission of idiopathic nephrotic syndrome (INS) of varied histology in patients who do not respond to the conventional treatment. This study was done to assess the safety and efficacy of methyl prednisolone and chlorambucil regimen in patients with various types of glomerulonephritides which were resistant to the usual conventional immunosuppressive drugs. Thirty nine patients were treated between June 1998 and December 2003 with Ponticelli regimen for six months. Twenty three patients (58.98%) were men and 16 (41.02%) were women. Mean age at the onset of NS was 23.59 +/- 1.28 (range 10-51) years. Four patients (10.2%) had minimal change disease (MCD), six patients (15.4%) had membranoproliferative glomerulonephritis (MPGN), two (5.1%) had IgA nephropathy, and 18 patients (46.1%) had focal segmental glomerulosclerosis (FSGS). Eleven patients were excluded from the final analysis. Of the remaining 28 patients, mean baseline proteinuria was 3.31 +/- 3.09 g/day. Mean baseline plasma albumin was 2.84 +/- 1.002 g/dl and mean baseline serum creatinine was 0.87 +/- 0.42 mg/dl. At the end of six months of treatment, mean proteinuria was 1.02 +/- 0.85 g/day. Mean plasma albumin was 3.69 +/- 0.78 g/day, and mean serum creatinine was 0.85 +/- 0.26 mg/dl. Mean followup was 13.21 +/- 7.7 times in 18.92 +/- 12.58 months. At the end of six months of treatment, seven patients (25%) achieved complete remission (CR), 10 patients (35.71%) partial remission (PR), and 11 patients (39.3%) did not show any response to the therapy. Most of the patients in responder group had FSGS (64.70%), whereas in nonresponder group patients had MPGN and mesangioproliferative glomerulonephritis (MesPGN). Out of 13 FSGS cases five (38.46%) achieved CR, six (46.15%) PR, and only two (15.38%) failed to respond. The incidence of side effects was 39.3%. Responders had more side effects than nonresponders (47 vs 27.3%). Methyl prednisolone and chlorambucil therapy (Ponticelli regimen) is safe and efficacious in achieving remission in significant number of INS patients other than membranous nephropathy, without any serious side effect on short term followup. However, a longer followup is required to demonstrate the sustained efficacy and long-term side effect of this regimen.
多项研究表明,对于常规治疗无反应的患者,使用甲泼尼龙和苯丁酸氮芥进行治疗可增加不同组织学类型的特发性肾病综合征(INS)缓解的机会。本研究旨在评估甲泼尼龙和苯丁酸氮芥方案对各种类型对常用常规免疫抑制药物耐药的肾小球肾炎患者的安全性和有效性。1998年6月至2003年12月期间,39例患者接受了Ponticelli方案治疗6个月。23例患者(58.98%)为男性,16例(41.02%)为女性。肾病综合征发病时的平均年龄为23.59±1.28岁(范围10 - 51岁)。4例患者(10.2%)为微小病变肾病(MCD),6例患者(15.4%)为膜增生性肾小球肾炎(MPGN),2例(5.1%)为IgA肾病,18例患者(46.1%)为局灶节段性肾小球硬化(FSGS)。11例患者被排除在最终分析之外。其余28例患者中,平均基线蛋白尿为3.31±3.09g/天。平均基线血浆白蛋白为2.84±1.002g/dl,平均基线血清肌酐为0.87±0.42mg/dl。治疗6个月结束时,平均蛋白尿为1.02±0.85g/天。平均血浆白蛋白为3.69±0.78g/天,平均血清肌酐为0.85±0.26mg/dl。平均随访18.92±12.58个月,随访次数为13.21±7.7次。治疗6个月结束时,7例患者(25%)达到完全缓解(CR),10例患者(35.71%)部分缓解(PR),11例患者(39.3%)对治疗无反应。反应组中的大多数患者为FSGS(64.70%),而无反应组患者为MPGN和系膜增生性肾小球肾炎(MesPGN)。13例FSGS病例中,5例(38.46%)达到CR,6例(46.15%)PR,仅2例(15.38%)无反应。副作用发生率为39.3%。反应者的副作用比无反应者更多(47%对27.3%)。甲泼尼龙和苯丁酸氮芥治疗(Ponticelli方案)对于除膜性肾病外的大量INS患者实现缓解是安全有效的,短期随访无任何严重副作用。然而,需要更长时间的随访来证明该方案的持续疗效和长期副作用。