Long-term renal outcomes of childhood-onset global and segmental diffuse proliferative lupus nephritis

Rianthavorn, Pornpimol; Buddhasri, Athitaya

doi:10.1007/s00467-015-3138-y

Cited by 30 publications

(15 citation statements)

References 36 publications

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“…The occurrence of the classes of LN in adults and children at new diagnosis is identical (19). The utility of distinguishing between histological classes of LN, such as proliferative and membranous types, and between segmental and global forms of proliferative LN is supported by studies of cSLE (20)(21)(22)(23).…”

Section: Advances In Interpretation Of Kidney Biopsies For Lupus Nephmentioning

confidence: 99%

“…The management change associated temporally with the largest improvement in renal survival was the addition of maintenance immunosuppression after induction therapy (52). Despite immunosuppression, only 55% of cSLE patients with proliferative LN (class III and IV) achieve renal remission (22,86,87). While 90% of cSLE patients with class V LN in cSLE achieve renal remission, only 76% can maintain remission despite low dose oral corticosteroids and/or maintenance immunosuppression such as AZA or MMF (88,89).…”

Section: Outcomes Of Lupus Nephritis In Children In 2016mentioning

confidence: 99%

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Advances in the care of children with lupus nephritis

2016

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The care of children with lupus nephritis (LN) has changed dramatically over the past 50 y. The majority of patients with childhood-onset systemic lupus erythematosus (cSLE) develop LN. In the 1960's, prognosis in children was worse than in adults; therapies were limited and toxic. Nearly half of cases resulted in death within 2 y. Since this time, several diagnostic recommendations and disease-specific indices have been developed to assist physicians caring for patients with LN. Pediatric researchers are validating and adapting these indices and guidelines for the treatment of LN in cSLE. Classification systems, activity, and chronicity indices for kidney biopsy have been validated in pediatric cohorts in several countries. Implementation of contemporary immunosuppressive agents has reduced treatment toxicity and improved outcomes. Biomarkers sensitive to LN in children have been identified in the kidney, urine, and blood. Multi-institutional collaborative networks have formed to address the challenges of pediatric LN research. Considerable variation in evaluation and treatment has been addressed for proliferative forms of LN by development of consensus treatment practices. Patient survival at 5 y is now 95-97% and renal survival exceeds 90%. Moreover, international consensus exists for quality indicators for cSLE that consider the unique aspects of chronic disease in childhood.

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Section: Advances In Interpretation Of Kidney Biopsies For Lupus Nephmentioning

confidence: 99%

Section: Outcomes Of Lupus Nephritis In Children In 2016mentioning

confidence: 99%

Advances in the care of children with lupus nephritis

2016

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“…Systemic lupus erythematosus (SLE) is a multisystem inflammatory autoimmune disease, and lupus nephritis (LN) is one of the main determinants of poor prognosis 1,2,3,4 . Although data from large-scale epidemiological studies are lacking, an estimated 10% of the children and adolescents will develop endstage renal disease (ESRD) within 10 years of LN diagnosis 5 , and 22% of children in ESRD from LN will die within 5 years of requiring renal replacement therapy 6 .…”

mentioning

confidence: 99%

Urine Biomarkers to Predict Response to Lupus Nephritis Therapy in Children and Young Adults

Brunner

Bennett²,

Gulati³

et al. 2017

J Rheumatol

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“…The spectrum of kidney disease in SLE ranges from hematuria and proteinuria to diffuse proliferative glomerulonephritis leading to chronic kidney disease (CKD) and end stage renal disease (ESRD) [1,3]. Despite immunosuppressive therapy, only slightly more than half of patients with childhood-onset SLE who develop LN with proliferative glomerular lesions achieve a renal remission, leaving them at high risk for the development of ESRD [4–6]. The development of LN in children with SLE is also associated with significantly increased risk for long-term morbidity and mortality.…”

Section: Introductionmentioning

confidence: 99%

Hepcidin and arterial stiffness in children with systemic lupus erythematosus and lupus nephritis: A cross-sectional study

2019

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Background Cardiovascular disease is common in patients with systemic lupus erythematosis (SLE) and lupus nephritis (LN). Up to 80% of children with SLE develop kidney disease, which is also associated with increased risk for cardiovascular disease and death compared to those without renal involvement. Hepcidin is an iron-regulatory protein which may contribute to atherosclerosis and is elevated in autoimmune disease. Pulse wave velocity (PWV) is a validated indicator of arterial stiffness, an early marker of cardiovascular risk, and is increased in children with SLE versus healthy controls. Our objective was to quantify hepcidin and PWV in children with SLE and investigate if those with biopsy-proven LN have higher hepcidin levels and higher PWV compared to those without kidney disease. Methods Cross-sectional analysis with hepcidin was measured via ELISA assay in 16 children aged 10–21 years with SLE recruited from a single center. Subjects were classified as having LN if histologic evidence of the disease was documented on a clinical renal biopsy. Serum hepcidin was quantified using a validated competitive enzyme-linked immunoassay. Carotid-femoral PWV was measured using applanation tonometry. Wilcoxon rank sum testing was used to compare median levels of hepcidin, PWV, and other continuous variables by nephritis status. Results The cohort (n = 16) was 93.8% female and 68.8% African-American with mean (SD) 16 (3.6) years. 37.5% (n = 6) had LN. Overall median (IQR) hepcidin was 34.4 (18.9, 91.9) ng/ml, and PWV 4.4 (4, 4.6) meters/second. Although significance was limited by small sample size, both hepcidin and PWV were higher in the subjects with LN. Median (IQR) hepcidin in subjects with LN was 71.5 (26.4, 116.4) ng/ml compared to 27.9 (18.7, 59.7) ng/ml in those with SLE(p = 0.19). Similarly, median (IQR) PWV in those with LN was 4.4 (4.4, 4.9) meters/second compared to 3 (3.75, 4.55) meters/second in those with without kidney involvement (p = 0.10). Conclusion PWV and serum hepcidin were higher in subjects with LN compared to those with SLE alone, suggesting that elevated hepcidin levels may be associated with morbid CV changes in children with LN. This association, along with identification of additional predictors of arterial stiffness in patients with LN, warrants further investigation.

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Long-term renal outcomes of childhood-onset global and segmental diffuse proliferative lupus nephritis

Abstract: Although patients with IV-G and IV-S displayed some clinical and histopathological disparities, renal outcomes were similar. The majority of children with DPLN reached adulthood but accrued significant renal damage. Treatment regimens which can slow the progression of CKD are needed.

Cited by 30 publications

References 36 publications

Advances in the care of children with lupus nephritis

Advances in the care of children with lupus nephritis

Urine Biomarkers to Predict Response to Lupus Nephritis Therapy in Children and Young Adults

Hepcidin and arterial stiffness in children with systemic lupus erythematosus and lupus nephritis: A cross-sectional study

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