Faedda R, Palomba D, Satta A, Pirisi M, Tanda F, Bartoli E
Istituto di Patologia Medica, Università degli Studi, Sassari, Italy.
Clin Nephrol. 1995 Dec;44(6):367-75.
To evaluate the results, the long-term prognosis and the rates of complication of an immunosuppressive regimen with corticosteroids and cyclophosphamide in the treatment of the nephritis of systemic lupus erythematosus, 21 patients with lupus glomerulonephritis were studied. Renal biopsies were performed in 17/21 of them and indicated diffuse proliferative (6 patients), diffuse mesangial (4) and membranous (7) glomerulonephritis. Treatment was structured in 4 phases: 1) induction with methylprednisolone 250 mg i.v. for 7-14 days, and cyclophosphamide 100-200 mg p.o., q.d., or 20 mg/kg i.v. every 28 days; 2) maintenance with prednisone p.o., 2 mg/kg q.o.d. for 45 days, and cyclophosphamide as before; 3) tapering, with reduction of prednisone by 15% each month for 4 months; 4) indefinite maintenance with prednisone slowly tapered to the least effective q.o.d. dose and cyclophosphamide discontinued after six months of treatment. This cycle was repeated in the event of a relapse. After a first immunosuppressive cycle, 20/21 patients achieved remission of glomerulonephritis. Plasma creatinine fell from 97 +/- 6 to 80 +/- 3 microMol/l (p < 0.01). Proteinuria fell from 2.1 +/- 0.4 to 0.2 +/- 0.4 g/d (p < 0.0001) and the nephrotic syndrome, present in 8 patients, disappeared. After an average of 20 +/- 7 months, 8 patients relapsed: all remitted again after a repeat cycle, but 1 later progressed to end-stage renal failure during pregnancy. After an average of 56 months 4 out of these 8 patients relapsed again: 1 progressed to end-stage renal disease following an abortion and 3 remitted completely after a third cycle. Thus, 18 out of 21 patients are presently in remission with an average dose of prednisone of 13.7 mg/day after an average follow-up of 52 +/- 38 months (range 2 to 156). Three patients are presently off treatment. In 16 patients with extended follow-up of 2 to 13 years, anti-nuclear antibodies, anti-DNA antibodies, albuminuria and cylindruria fell below post-cycle levels (p < 0.001 for all). We conclude that intensive immunosuppression with steroids and cyclophosphamide can achieve excellent long-term results in the treatment of systemic lupus with glomerulonephritis.
为评估皮质类固醇和环磷酰胺免疫抑制方案治疗系统性红斑狼疮肾炎的效果、长期预后及并发症发生率，对21例狼疮性肾小球肾炎患者进行了研究。其中17/21例患者进行了肾活检，结果显示为弥漫性增殖性肾小球肾炎（6例）、弥漫性系膜增生性肾小球肾炎（4例）和膜性肾小球肾炎（7例）。治疗分为4个阶段：1）诱导期，静脉注射甲泼尼龙250mg，持续7 - 14天，口服环磷酰胺100 - 200mg，每日1次，或每28天静脉注射20mg/kg；2）维持期，口服泼尼松，2mg/kg，隔日1次，共45天，环磷酰胺用法同前；3）减量期，泼尼松每月减量15%，共4个月；4）长期维持期，泼尼松缓慢减量至最低有效隔日剂量，治疗6个月后停用环磷酰胺。复发时重复此周期。经过第一个免疫抑制周期后，20/21例患者的肾小球肾炎得到缓解。血浆肌酐从97±6降至80±3微摩尔/升（p<0.01）。蛋白尿从2.1±0.4降至0.2±0.4克/天（p<0.0001），8例患者存在的肾病综合征消失。平均20±7个月后，8例患者复发：再次进行一个周期治疗后均再次缓解，但1例患者在妊娠期间进展为终末期肾衰竭。平均56个月后，这8例患者中有4例再次复发：1例在流产后进展为终末期肾病，3例在第三个周期后完全缓解。因此，21例患者中有18例目前处于缓解状态，平均泼尼松剂量为13.7mg/天，平均随访52±38个月（范围2至156个月）。3例患者目前已停止治疗。在16例随访2至13年的患者中，抗核抗体、抗DNA抗体、蛋白尿和管型尿均降至周期后的水平以下（所有p<0.001）。我们得出结论，类固醇和环磷酰胺强化免疫抑制治疗系统性红斑狼疮合并肾小球肾炎可取得优异的长期效果。
