Clinicopathological characteristics of pediatric ANCA-associated glomerulonephritis.

Autor:	Charnaya, Olga, Kruglyakova, Jacqueline, Jacob, Binil, Arend, Lois J.
Předmět:	VASCULITIS KIDNEY failure RISK assessment BIOPSY PROTEINURIA ANTINEUTROPHIL cytoplasmic antibodies SEX distribution RARE diseases TREATMENT effectiveness RETROSPECTIVE studies MULTIVARIATE analysis GLOMERULONEPHRITIS DISEASES MEDICAL records ACQUISITION of data PLASMA exchange (Therapeutics) CYCLOPHOSPHAMIDE KIDNEYS REGRESSION analysis DISEASE risk factors DISEASE complications CHILDREN
Zdroj:	Pediatric Nephrology; Oct2024, Vol. 39 Issue 10, p2947-2957, 11p
Abstrakt:	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. 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. 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. 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. [ABSTRACT FROM AUTHOR]
Databáze:	Complementary Index
Externí odkaz:	Zobrazit plný text záznamu Full text from SpringerLink