Long-term follow-up of aggressively treated idiopathic rapidly progressive glomerulonephritis.
OBJECTIVE
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.


METHODS
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.


RESULTS
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.


CONCLUSIONS
Thus, long remissions are seen in most patients with RPGN treated aggressively.
Bruns
F J
FJ
Department of Medicine, University of Pittsburgh School of Medicine, Pennsylvania.
Adler
S
S
Fraley
D S
DS
Segel
D P
DP
D000328:Adult; D000368:Aged; D000936:Antigen-Antibody Complex / Q000032:analysis; D001323:Autoantibodies / Q000032:analysis; D003131:Combined Modality Therapy; D003404:Creatinine / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000188:drug therapy / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology; D007678:Kidney Glomerulus / Q000276:immunology; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D010956:Plasmapheresis; D011241:Prednisone / Q000627:therapeutic use
D000936:Antigen-Antibody Complex; D001323:Autoantibodies; D003520:Cyclophosphamide; D003404:Creatinine; D011241:Prednisone; D008775:Methylprednisolone