Bruns F J, Adler S, Fraley D S, Segel D P
Department of Medicine, University of Pittsburgh School of Medicine, Pennsylvania.
Am J Med. 1989 Apr;86(4):400-6. doi: 10.1016/0002-9343(89)90336-7.
We wanted to examine the long-term effects of aggressively treating idiopathic rapidly progressive glomerulonephritis (RPGN), with a particular focus on clinically characterizing the patient population, assessing the short- and long-term effects of therapy on renal function, and determining complications of the therapy.
Twenty-three consecutive patients with RPGN were treated and followed from one to 11 years. On renal biopsy, 13 had immune complexes, eight had no immune complexes, and two had antiglomerular basement membrane deposits. All had greater than 25 percent crescents and 19 of 23 had greater than 50 percent crescents. Every patient responded on a short-term basis to either large-dose pulse methylprednisolone or plasma exchange, with reduction of the mean plasma creatinine level from 6.5 +/- 2.0 mg/dl to 2.9 +/- 1.0 mg/dl (p less than 0.001). Each patient received oral prednisone and all but one received cyclophosphamide.
Three died of non-renal causes. Fifty percent of the remaining 20 patients maintained stable renal function for at least two years. Four of nine patients followed-up for longer than two years had a relapse, but all responded again to therapy. No characteristic clinical symptoms predicting relapse were found, although nearly all had hematuria and proteinuria. Complications of therapy were frequent and may have contributed to death in two patients.
Thus, long remissions are seen in most patients with RPGN treated aggressively.
我们想要研究积极治疗特发性快速进展性肾小球肾炎（RPGN）的长期效果，特别关注对患者群体进行临床特征描述，评估治疗对肾功能的短期和长期影响，并确定治疗的并发症。
连续23例RPGN患者接受治疗并随访1至11年。肾活检显示，13例有免疫复合物，8例无免疫复合物，2例有抗肾小球基底膜沉积物。所有患者新月体均超过25%，23例中有19例新月体超过50%。每位患者短期内对大剂量脉冲甲基强的松龙或血浆置换均有反应，平均血浆肌酐水平从6.5±2.0mg/dl降至2.9±1.0mg/dl（p<0.001）。每位患者均接受口服强的松，除1例患者外均接受环磷酰胺治疗。
3例死于非肾性原因。其余20例患者中有50%肾功能至少维持稳定两年。随访超过两年的9例患者中有4例复发，但均再次对治疗有反应。尽管几乎所有患者都有血尿和蛋白尿，但未发现预测复发的特征性临床症状。治疗并发症很常见，可能导致2例患者死亡。
因此，积极治疗的大多数RPGN患者可实现长期缓解。
