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BackgroundAlthough evidence has con rmed that cyclosporine A (CsA) is e cacious against childhood-onset steroid-dependent and steroid-resistant nephrotic syndrome (SD/SRNS), some patients may continue to relapse during adulthood. However, predictive factors for active disease into adulthood and renal complications in this cohort remain unknown. MethodsWe conducted a retrospective study on the long-term outcomes of 81 young adults with childhood-onset SD/SRNS treated with CsA. The primary endpoint was the probability of active disease into adulthood, de ned as disease relapse or ongoing immunosuppressive treatment throughout the 2 years preceding the last follow-up. ResultsAt last follow-up (median age, 23.2 years; median disease duration, 15.8 years), 44 adult patients (54%) continued to have active disease, whereas 16 (20%) and 16 (20%) patients developed mild chronic kidney disease (CKD) and hypertension, respectively. Young age at NS onset and history of relapse during initial CsA treatment were independent predictive factors for active disease into adulthood. Acute kidney injury at NS onset, focal segmental glomerulosclerosis, and irreversible CsA nephrotoxicity were identi ed as risk factors for the development of CKD, whereas older age was identi ed as a risk factor for the development of renal complications. No correlation was observed between active disease into adulthood and the development of renal complications. ConclusionsAfter CsA initiation for SD/SRNS, more than half of adult survivors continued to have active disease.Long-term follow-up is necessary to identify the development of renal complications later in adulthood that can be attributed to prior disease and CsA treatment in childhood, irrespective of disease activity.
BackgroundAlthough evidence has con rmed that cyclosporine A (CsA) is e cacious against childhood-onset steroid-dependent and steroid-resistant nephrotic syndrome (SD/SRNS), some patients may continue to relapse during adulthood. However, predictive factors for active disease into adulthood and renal complications in this cohort remain unknown. MethodsWe conducted a retrospective study on the long-term outcomes of 81 young adults with childhood-onset SD/SRNS treated with CsA. The primary endpoint was the probability of active disease into adulthood, de ned as disease relapse or ongoing immunosuppressive treatment throughout the 2 years preceding the last follow-up. ResultsAt last follow-up (median age, 23.2 years; median disease duration, 15.8 years), 44 adult patients (54%) continued to have active disease, whereas 16 (20%) and 16 (20%) patients developed mild chronic kidney disease (CKD) and hypertension, respectively. Young age at NS onset and history of relapse during initial CsA treatment were independent predictive factors for active disease into adulthood. Acute kidney injury at NS onset, focal segmental glomerulosclerosis, and irreversible CsA nephrotoxicity were identi ed as risk factors for the development of CKD, whereas older age was identi ed as a risk factor for the development of renal complications. No correlation was observed between active disease into adulthood and the development of renal complications. ConclusionsAfter CsA initiation for SD/SRNS, more than half of adult survivors continued to have active disease.Long-term follow-up is necessary to identify the development of renal complications later in adulthood that can be attributed to prior disease and CsA treatment in childhood, irrespective of disease activity.
No abstract
Objectives There is little data on renal relapse in childhood-onset lupus nephritis (cLN). We investigate the incidence, predictive factors and outcomes related to renal relapse. Method We conducted a retrospective cohort study of all cLN diagnosed ≤18 years, between 2001–2021, to investigate the incidence and outcomes related to renal relapse. Results 95 Chinese cLN patients (91% proliferative LN) were included. Induction immunosuppression was prednisolone and cyclophosphamide (n = 36, 38%) or mycophenolate mofetil (MMF, n = 33, 35%). Maintenance immunosuppression was prednisolone and MMF (n = 53, 54%) or azathioprine (AZA, n = 29, 31%). The rates of complete/partial remission at 12-month were 78.9%/7.4%. 70 renal relapses occurred in 39 patients over a follow-up of 10.2 ± 5.9 years (0.07 episode per patient-year). Relapse-free survival was 94.7%, 86.0%, 80.1%, 71.2%, 68.3%, 50.3%, and 44.5% at 1-, 2-, 3-, 4-, 5-, 10-, and 20-year, respectively. Multivariate analysis showed that LN diagnosis <13.1 years (HRadj 2.59, 95% CI 1.27–5.29, p= 0.01), AZA maintenance (HRadj 2.20, 95% CI 1.01–4.79, p= 0.05), partial remission (HRadj 3.9, 95% CI 1.03–9.19, p= 0.01) and non-remission (HRadj 3.08, 95% CI 1.35–11.3, p= 0.04) at 12-month were predictive of renal relapse. Renal relapse was significantly associated with advanced chronic kidney disease (stage 3–5) and end-stage kidney disease (17.9% vs 1.8%, p< 0.01). Furthermore, patients with renal relapse(s) showed increased incidence of infections (30.8% vs 10.7%, p= 0.02), osteopenia (38.5% vs 17.9%, p= 0.04) and hypertension (30.8% vs 7.1%, p< 0.01). Conclusion Renal relapse is common among cLN especially among young patients, and is associated with increased incidence of morbidity and mortality. Attaining complete remission and the use of MMF appear to have reduced the incidence of renal relapses.
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