Over the past 50 years, survival has improved in patients with systemi
c lupus erythematosus and associated nephritis. Yet, there are few lon
g-term outcome studies in patients with well-defined nephropathy. We e
xaminbed the outcome of 439 patients with lupus nephritis who were see
n at the Mayo Clinic between 1964 and 1986 in whom renal biopsies were
assessed using the World Health Organization (WHO) classification. Th
ere were 341 women and 98 men (mean +/- s.d., age 33.5 +/- 14 years);
200 (46%) patients were hypertensive and 249 (57%) had impaired renal
function at renal biopsy. All WHO morphologic classes were represented
and 339 (77%) patients had class III, IV and V (the more severe forms
of nephritis). Follow-up averaged 10.2 years per patient. At last con
tact, 286 (65%) patients were alive and 153 (35%) were dead. Overall p
atient survival was 80%, 69% and 53% at 5, 10 and 20 years after biops
y that was significantly worse than expected survival (P < 0.001). Ten
-year cumulative patient survival improved comparing earlier to more r
ecent time spans: 64% in 231 patients seen during 1964-75; 76% in 2089
patients studied during 1976-86 (P = 0.03). Survival free of renal fa
ilure was 83%, 74% and 64% at 5, 10 and 20 years, and survival was unf
avorably influenced by progressive WHO class, hypertension, impaired r
enal function, nephrotic range proteinuria, hypoalbuminemia and anemia
. Multivariate analysis found impaired renal function, increased urine
protein, anemia and younger age to be independent predictors of renal
failure. WHO class was not a significant predictor when adjusted for
these four factors. Cardiovascular events accounted for 48% of the kno
wn deaths and were equally distributed across all WHO classes, followe
d by infections, renal failure, malignancy, respiratory failure and ga
strointestinal bleeding. Severe nephritis, nephrotic syndrome, hyperte
nsion and anemia remain strong predictors of a poor renal outcome. Pro
spective randomized studies stratifying for these factors are needed t
o find optimal immunosuppressive regimens to improve renal survival.