Steen VD (Introduction by Verbalis JG)
Outcome of scleroderma renal crisis (SRC).
ASN 30th Annual Meeting, San Antonio
J Am Soc Nephrol (Sep) 8:149A 1997
The natural history of scleroderma renal crisis has been dramatically altered by the use of ACE inhibitors. In this abstract the author presents data on 130 patients treated with these drugs. They were retrospectively divided into 4 groups:
- Early deaths: 23% (28 patients). Most required dialysis, and those dying had heart disease and were older than those who survived.
- Never required dialysis: 35% (46 patients). Most had malignant hypertension with azotemia during the intial episode, but never required dialysis. Although SCr peaked at 3.8, at long term follow-up SCr averaged 1.8 mg/dl.
- Required dialysis: 54% (29 patients). What is remarkable about this patient group is, that more than half were able to come off dialysis within 2 years. ACE inhibitors were continued throughout the time on dialysis (at a reduced dose).
- Relapse: 2 patients required dialysis after 3 and 6 years due to relapse of malignant hypertension.
Comment: The aggressive use of ACE inhibitors has changed the previously reported dismal course of scleroderma renal crisis with an ultimate outcome in renal failure and death. The author is to be commended for a provocative retrospective analysis of the largest cohort of scleroderma patients in the US. It appears to be important to continue the ACE inhibitors even on dialysis. (John Ball, M.D., Illinois Masonic Hospital, Chicago, IL)
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ASN 30th Annual Meeting, San Antonio
H: Pathophysiology : Kidney in hypertension