Etiology and Outcome of Crescentic Glomerulonephritis.
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Date
2013-03
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Abstract
Objective: To determine the etiology, course and predictors of
outcome in children with crescentic glomerulonephritis (GN).
Study design: Retrospective, descriptive study.
Setting: Pediatric Nephrology Clinic at a referral center in
Northern India.
Methods: Clinic records of patients aged <18 year with crescentic
GN diagnosed from 2001-2010 and followed at least 12-months
were reviewed. Crescentic GN, defined as crescents in ≥50%
glomeruli, was classified based on immunofluorescence findings
and serology. Risk factors for renal loss (chronic kidney disease
stage 4-5) were determined.
Results: Of 36 patients, (median age 10 yr) 17 had immune
complex GN and 19 had pauci-immune crescentic GN. The
etiologies of the former were lupus nephritis (n=4), postinfectious
GN (3), and IgA nephropathy, Henoch Schonlein purpura and
membranoproliferative GN type II (2 each). Three patients with
pauci-immune GN showed antineutrophil cytoplasmic antibodies
(ANCA). Rapidly progressive GN was present in 33 patients, and
required dialysis in 12. At median 34 (19-72) months, 2 patients
with immune complex GN and 8 with pauci-immune GN showed
renal loss. Renal survival was 94.1% at 3 yr, and 75.3% at 8 yr in
immune complex GN; in pauci-immune GN survival was 63.2%
and 54.1%, respectively (P=0.054). Risk factors for renal loss
were oliguria at presentation (hazards ratio, HR 10.50; P=0.037)
and need for dialysis (HR 6.33; P=0.024); there was inverse
association with proportion of normal glomeruli (HR 0.91;
P=0.042).
Conclusions: Pauci-immune GN constitutes one-half of patients
with crescentic GN at this center. Patients with pauci-immune GN,
chiefly ANCA negative, show higher risk of disease progression.
Renal loss is related to severity of initial presentation and extent of
glomerular involvement.
Description
Keywords
Antineutrophil cytoplasmic antibody, Rapidly progressive glomerulonephritis, Vasculitis
Citation
Sinha Aditi, Puri Kriti, Hari Pankaj, Dinda Amit Kumar, Bagga Arvind. Etiology and Outcome of Crescentic Glomerulonephritis. Indian Pediatrics. 2013 March; 50(3): 283-288.