Etiology and outcome of crescentic glomerulonephritis. 2013

Aditi Sinha, and Kriti Puri, and Pankaj Hari, and Amit Kumar Dinda, and Arvind Bagga
Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

OBJECTIVE To determine the etiology, course and predictors of outcome in children with crescentic glomerulonephritis (GN). METHODS Retrospective, descriptive study. METHODS 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 pauciimmune 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 pauciimmune GN showed renal loss. Renal survival was 94.1% at 3 yr, and 75.3% at 8 yr in immune complex GN; in pauciimmune 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 pauciimmune GN, chiefly ANCA negative, show higher risk of disease progression. Renal loss is related to severity of initial presentation and extent of glomerular involvement.

UI MeSH Term Description Entries
D007668 Kidney Body organ that filters blood for the secretion of URINE and that regulates ion concentrations. Kidneys
D007677 Kidney Function Tests Laboratory tests used to evaluate how well the kidneys are working through examination of blood and urine. Function Test, Kidney,Function Tests, Kidney,Kidney Function Test,Test, Kidney Function,Tests, Kidney Function
D008297 Male Males
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005260 Female Females
D005921 Glomerulonephritis Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY. Bright Disease,Kidney Scarring,Glomerulonephritides,Scarring, Kidney
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective
D053208 Kaplan-Meier Estimate A nonparametric method of compiling LIFE TABLES or survival tables. It combines calculated probabilities of survival and estimates to allow for observations occurring beyond a measurement threshold, which are assumed to occur randomly. Time intervals are defined as ending each time an event occurs and are therefore unequal. (From Last, A Dictionary of Epidemiology, 1995) Kaplan-Meier Survival Curve,Kaplan-Meier Analysis,Kaplan-Meier Survival Curves,Kaplan-Meier Test,Product-Limit Method,Analysis, Kaplan-Meier,Curve, Kaplan-Meier Survival,Curves, Kaplan-Meier Survival,Estimate, Kaplan-Meier,Kaplan Meier Analysis,Kaplan Meier Survival Curve,Kaplan Meier Survival Curves,Kaplan Meier Test,Method, Product-Limit,Methods, Product-Limit,Product Limit Method,Product-Limit Methods,Survival Curve, Kaplan-Meier,Survival Curves, Kaplan-Meier,Test, Kaplan-Meier
D056648 Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Group of systemic vasculitis with a strong association with ANCA. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls. ANCA-Associated Vasculitides,ANCA-Associated Vasculitis,Pauci-Immune Vasculitis,ANCA Associated Vasculitides,ANCA Associated Vasculitis,ANCA-Associated Vasculitide,Anti Neutrophil Cytoplasmic Antibody Associated Vasculitis,Pauci Immune Vasculitis,Pauci-Immune Vasculitides,Vasculitide, ANCA-Associated,Vasculitides, ANCA-Associated,Vasculitides, Pauci-Immune,Vasculitis, ANCA-Associated,Vasculitis, Pauci-Immune

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