Renal vascular lesions in childhood-onset lupus nephritis.

Autor: Lin KY; Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong, SAR., Chan EY; Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong, SAR. genegene.chan@gmail.com.; Department of Paediatrics, The Chinese University of Hong Kong, Hong Kong, Hong Kong, SAR. genegene.chan@gmail.com., Mak YF; Department of Pathology, Princess Margaret Hospital, Hong Kong, Hong Kong, SAR., To MC; Department of Pathology, Princess Margaret Hospital, Hong Kong, Hong Kong, SAR., Wong SW; Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong, SAR., Lai FF; Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong, SAR., Ho TW; Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong, SAR., Tong PC; Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong, SAR., Lai WM; Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong, SAR., Yap DY; Division of Nephrology, Department of Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, Hong Kong, SAR., Ma AL; Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong, SAR.; Department of Paediatrics and Adolescent Medicine, The University of Hong Kong, Hong Kong, Hong Kong, SAR.
Jazyk: angličtina
Zdroj: Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2025 Jan; Vol. 40 (1), pp. 131-141. Date of Electronic Publication: 2024 Sep 09.
DOI: 10.1007/s00467-024-06498-z
Abstrakt: Background: This study aimed to determine the clinical significance of renal vascular lesions (RVLs) in childhood-onset lupus nephritis (cLN).
Methods: We retrospectively reviewed all children with biopsy-proven cLN between 2004-2020 to evaluate the prevalence of RVLs on kidney biopsy and its associated factors and long-term outcomes. The composite kidney outcome was defined as advanced chronic kidney disease (CKD) stage 3-5, kidney failure and death.
Results: 107 biopsies from 84 Chinese patients were analysed. RVLs were observed in 19 patients (22.6%), including non-inflammatory necrotizing vasculopathy (NNV, n = 6), thrombotic microangiopathy (TMA, n = 4), arterial sclerosis (AS, n = 3), concurrent NNV with AS (n = 4), concurrent NNV with TMA (n = 1) and concurrent true renal vasculitis with AS (n = 1). The presence of RVLs was associated with lower estimated glomerular filtration rate (eGFR) (66.9 ± 40.3 vs. 95.6 ± 39.4 ml/min/1.73m 2 , p = 0.005), haemoglobin level (9.1 ± 1.9 vs. 10.4 ± 1.9 g/dL, p = 0.008) and platelet count (150.1 ± 96.4 vs. 217.2 ± 104.8 × 10 9 /L, p = 0.01). LN classes and activity/chronicity indices were similar. Patients with RVLs had poorer composite kidney outcomes, though not reaching statistical significance (log-rank test, p = 0.06). The presence of NNV was associated with inferior survival free from composite kidney outcome (log-rank test, p = 0.0018), compared to other forms of RVLs and those without RVLs. Univariate analysis revealed NNV (HR 7.08, 95% CI 1.67-30.03) was predictive of composite kidney outcome.
Conclusion: RVLs are present in one-fifth of cLN patients and are associated with severe presentation. NNV is associated with worse long-term kidney outcome. Routine evaluation of RVLs is warranted and should be incorporated into future classification criteria.
Competing Interests: Declarations. Conflict of interest: The authors declare that they have no conflict of interest.
(© 2024. The Author(s).)
Databáze: MEDLINE
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