Managing Lupus Nephritis in Children and Adolescents

Chan, Eugene Yu-hin; Lai, Fiona Fung-yee; Ma, Alison Lap-tak; Chan, Tak Mao

doi:10.1007/s40272-023-00609-3

Cited by 3 publications

(2 citation statements)

References 118 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…[1,2] Around 4% of cSLE developed advanced chronic kidney disease (CKD). [2,3] Up to 15% of patients with childhood-onset lupus nephritis (cLN) may progress to end-stage kidney disease (ESKD), [4,5] and is also associated with signi cant morbidity and mortality, compared to general paediatric population. [6] Histopathological ndings in LN are de ned according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classi cation.…”

Section: Introductionmentioning

confidence: 99%

“…[9,10] cLN shows substantial differences in the disease pathogenesis, clinical presentation, epidemiology and outcomes compared to adult-onset LN. [5] There is also no currently available data pertaining to the clinical signi cance of RVLs in cLN. In view of such knowledge gap, this study was set forth to investigate the prevalence, associated factors and long-term outcomes of RVLs in biopsy-proven cLN.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Renal vascular lesions in Childhood-onset Lupus Nephritis

Lin,

Chan,

Mak

et al. 2024

Preprint

Self Cite

View full text Add to dashboard Cite

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 renal outcome was defined as advanced chronic kidney disease (CKD) stage 3-5, end-stage kidney disease (ESKD) 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±39.4ml/min/1.73m2, p=0.005), haemoglobin level (9.1±1.9 vs 10.4±1.9g/dL, p=0.008) and platelet count (150.1±96.4 vs 217.2±104.8 x 109/L, p=0.01). LN classes and activity/chronicity indices were similar. Patients with RVLs had poorer composite renal outcomes, though not reaching statistical significance (log-rank test, p=0.06). The presence of NNV was associated with inferior survival free from composite renal 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 renal outcome. Conclusion: RVLs are present in one-fifth of cLN patients and are associated with severe presentation. NNV is associated with worse long-term renal outcome. Routine evaluation of RVLs is warranted and should be incorporated into future classification criteria.

show abstract

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Renal vascular lesions in Childhood-onset Lupus Nephritis

Lin,

Chan,

Mak

et al. 2024

Preprint

Self Cite

View full text Add to dashboard Cite

show abstract

Do we really need cyclophosphamide for lupus nephritis?

Wenderfer,

Cooper

2024

Pediatr Nephrol

View full text Add to dashboard Cite

Systemic lupus erythematosus with juvenile onset: current status of the problem (literature review)

Kaleda,

Nikishina

2024

Sovremennaâ revmatologiâ

View full text Add to dashboard Cite

Patients with juvenile-onset systemic lupus erythematosus (jSLE) account for up to 25% of all SLE patients. The main difference between jSLE and SLE in adults is the greater role of genetic factors in the pathogenesis, higher activity, earlier development of complications and the need for more aggressive immunosuppressive therapy, which allows us to consider the onset of the disease in childhood as a special phenotype of SLE. The relevance of the study of jSLE arises from the variability of clinical manifestations and the unpredictability of the course, the difficulty of early diagnosis, the rapid development of organ damage and the unfavorable life prognosis.The article presents the most important modern data on the diagnosis, classification, features of the clinical picture and treatment approaches of jSLE from a practical point of view.

show abstract

Managing Lupus Nephritis in Children and Adolescents

Cited by 3 publications

References 118 publications

Renal vascular lesions in Childhood-onset Lupus Nephritis

Renal vascular lesions in Childhood-onset Lupus Nephritis

Do we really need cyclophosphamide for lupus nephritis?

Systemic lupus erythematosus with juvenile onset: current status of the problem (literature review)

Contact Info

Product

Resources

About