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Clinicopathological characteristics of pediatric ANCA-associated glomerulonephritis.
- Source :
-
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2024 Oct; Vol. 39 (10), pp. 2947-2957. Date of Electronic Publication: 2024 May 23. - Publication Year :
- 2024
-
Abstract
- Background: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and glomerulonephritis is uncommon in children. We sought to characterize the histological and clinical features of the disease and report on risk factors for adverse outcomes in a pediatric cohort.<br />Methods: Retrospective single-center cohort of all pediatric (< 20 years) patients diagnosed with ANCA-associated glomerulonephritis (AAGN) by kidney biopsy between 2002 and 2022 at Johns Hopkins University. Histological and clinical features were extracted from the medical record. Clinical, laboratory, and histological findings were analyzed to determine the association with kidney failure (KF) and/or death.<br />Results: A total of 17 patients were identified (GPA n = 7, MPA = 10) with a median age of 15 years (IQR 12-17) at presentation, a slight female predominance (59%), with seven patients reaching the composite outcome of death (n = 1) or kidney failure (n = 6). There was no difference in presenting clinical symptoms or extra-renal manifestations between the two groups. Univariable Cox regression identified several factors associated with an increased hazard of endpoint including the degree of global or segmental sclerosis, interstitial fibrosis and tubular atrophy (IFTA), C3 and C1q staining, presence of subendothelial deposits, and proteinuria. Multivariable regression was not performed due to the small sample size. We saw a trend towards increased utilization of plasma exchange and a decrease in cyclophosphamide utilization in the more recent era. There was no association between treatment modality and outcome.<br />Conclusions: Pediatric AAGN is a rare disease associated with significant morbidity. We identified glomerulosclerosis and IFTA on histology, and proteinuria on initial presentation as risk factors for KF/death.<br /> (© 2024. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)
- Subjects :
- Humans
Female
Male
Retrospective Studies
Adolescent
Child
Risk Factors
Biopsy
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis complications
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis pathology
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis immunology
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis mortality
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis diagnosis
Kidney pathology
Kidney immunology
Antibodies, Antineutrophil Cytoplasmic blood
Antibodies, Antineutrophil Cytoplasmic immunology
Renal Insufficiency etiology
Immunosuppressive Agents therapeutic use
Proteinuria etiology
Glomerulonephritis pathology
Glomerulonephritis immunology
Glomerulonephritis mortality
Subjects
Details
- Language :
- English
- ISSN :
- 1432-198X
- Volume :
- 39
- Issue :
- 10
- Database :
- MEDLINE
- Journal :
- Pediatric nephrology (Berlin, Germany)
- Publication Type :
- Academic Journal
- Accession number :
- 38780769
- Full Text :
- https://doi.org/10.1007/s00467-024-06406-5