Περίληψη:
Background: Pauci-immune necrotizing glomerulonephritis (PING) occurs in
various settings and has a very variable prognosis. We investigated
whether clinicopathologic findings at the time of renal biopsy may
predict major disease outcomes. Methods: We evaluated 72 consecutive
patients with biopsy-documented PING. Kaplan-Meier curves and Cox models
assessed event rates and risk factors for death, end-stage renal disease
(ESRD), and death or new ESRD (after the renal biopsy). Results: During
a follow-up of 305 person-years, 11 patients died, 13 patients developed
ESRD, and 16 patients died or developed new ESRD. Among patients first
seen within 3 months of renal biopsy (incident cases), the 5-year
mortality rate was 20%, whereas the death or new ESRD rate was 34%. In
univariate analyses, older age, lower creatinine clearance, erythrocyte
sedimentation rate, and percentages of abnormal glomeruli, glomeruli
with fibrous crescents, and glomeruli with global sclerosis were
significant predictors of mortality, whereas antineutrophil cytoplasmic
autoantibodies with cytoplasmic staining conferred borderline
protection. For ESRD, significant predictors included a greater
creatinine level, lower hematocrit, interstitial fibrosis, tubular
necrosis, greater C-reactive protein level, and percentages of abnormal
glomeruli, glomeruli with extracapillary proliferation, cellular
crescents, and global glomerulosclerosis. For death or new ESRD,
predictors were fairly similar. Adjusting for baseline creatinine level,
the risk for ESRD increased 1.78-fold (95% confidence interval [CI],
1.23 to 2.58) per each 10% increase in global sclerosis and 1.47-fold
(95% CI, 1.05 to 2.07) per each 10% increase in glomeruli with
cellular crescents. Conclusion: Global glomerulosclerosis and crescents
in a renal biopsy are strong predictors of the long-term outcome of
PING.
Συγγραφείς:
Kapitsinou, PP
Ioannidis, JPA
Boletis, JN
Sotsiou, F and
Nakopoulou, L
Daphnis, E
Moutsopoulos, HM