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Glomerular Outgrowth as an Ex Vivo Assay to Analyze Pathways Involved in Parietal Epithelial Cell Activation
Published on: August 19, 2020
Etiology and outcome of crescentic glomerulonephritis
Aditi Sinha1, Kriti Puri, Pankaj Hari
1Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Insights
Pauci-immune crescentic glomerulonephritis (GN) is common in children and carries a higher risk of kidney damage. Early signs like oliguria and dialysis needs predict worse outcomes in pediatric crescentic GN.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Immunopathology
Background:
- Crescentic glomerulonephritis (GN) is a severe kidney disease characterized by rapid glomerular damage.
- Understanding the specific types, causes, and outcomes of crescentic GN in children is crucial for effective management.
Purpose of the Study:
- To investigate the causes, disease progression, and outcome predictors in pediatric patients diagnosed with crescentic glomerulonephritis.
- To classify crescentic GN based on immunofluorescence and serology and assess associated risk factors for renal failure.
Main Methods:
- A retrospective descriptive study was conducted on children (<18 years) with crescentic GN diagnosed between 2001-2010.
- Crescentic GN was defined as crescents in ≥50% of glomeruli; classification involved immunofluorescence and serology.
- Risk factors for renal loss (CKD stages 4-5) were analyzed.
Main Results:
- Out of 36 children, 17 had immune complex GN and 19 had pauci-immune crescentic GN.
- Pauci-immune GN patients, often ANCA-negative, showed a trend towards poorer renal survival (54.1% at 8 years) compared to immune complex GN (75.3% at 8 years).
- Oliguria at presentation and need for dialysis were significant predictors of renal loss, while a higher proportion of normal glomeruli was protective.
Conclusions:
- Pauci-immune GN accounts for half of pediatric crescentic GN cases in this cohort, presenting a greater risk for disease progression.
- Children with pauci-immune GN, particularly ANCA-negative cases, face a higher likelihood of experiencing renal loss.
- The severity of the initial presentation and the extent of glomerular damage are key determinants of renal outcomes in pediatric crescentic GN.
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 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.
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