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Long-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis

INTRODUCTION: Long-term data pertaining to childhood-onset lupus nephritis (cLN) remain extremely scarce. METHODS: We conducted a retrospective cohort study of biopsy-proven cLN with onset age <18 years diagnosed from 2001 to 2020 to ascertain the long-term patient and kidney survival rates, and...

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Autores principales: Chan, Eugene Yu-hin, Yap, Desmond Yat-hin, Wong, Wing-tung, Wong, Wilfred Hing-sang, Wong, Sze-wa, Lin, Kyle Ying-kit, Hui, Felix Yan-wai, Yee-ming, Jennifer, Lam, Sophia Suet-ying, Wong, Jennie Kit-yee, Lai, Fiona Fung-yee, Ho, Tsz-wai, Tong, Pak-chiu, Lai, Wai-ming, Chan, Tak Mao, Ma, Alison Lap-tak
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9831948/
https://www.ncbi.nlm.nih.gov/pubmed/36644360
http://dx.doi.org/10.1016/j.ekir.2022.10.014
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author Chan, Eugene Yu-hin
Yap, Desmond Yat-hin
Wong, Wing-tung
Wong, Wilfred Hing-sang
Wong, Sze-wa
Lin, Kyle Ying-kit
Hui, Felix Yan-wai
Yee-ming, Jennifer
Lam, Sophia Suet-ying
Wong, Jennie Kit-yee
Lai, Fiona Fung-yee
Ho, Tsz-wai
Tong, Pak-chiu
Lai, Wai-ming
Chan, Tak Mao
Ma, Alison Lap-tak
author_facet Chan, Eugene Yu-hin
Yap, Desmond Yat-hin
Wong, Wing-tung
Wong, Wilfred Hing-sang
Wong, Sze-wa
Lin, Kyle Ying-kit
Hui, Felix Yan-wai
Yee-ming, Jennifer
Lam, Sophia Suet-ying
Wong, Jennie Kit-yee
Lai, Fiona Fung-yee
Ho, Tsz-wai
Tong, Pak-chiu
Lai, Wai-ming
Chan, Tak Mao
Ma, Alison Lap-tak
author_sort Chan, Eugene Yu-hin
collection PubMed
description INTRODUCTION: Long-term data pertaining to childhood-onset lupus nephritis (cLN) remain extremely scarce. METHODS: We conducted a retrospective cohort study of biopsy-proven cLN with onset age <18 years diagnosed from 2001 to 2020 to ascertain the long-term patient and kidney survival rates, and the incidence of advanced chronic kidney disease (CKD) (estimated glomerular filtration rate [eGFR] <60 ml/min per 1.73 m(2)). RESULTS: A total of 92 subjects (78 female; age 13.7 ± 3.3 years; all Chinese) were included, with follow-up duration of 10.3 years (interquartile range, 5.8–15.9). Of these, 83 children (90%) had proliferative lupus nephritis (LN) (Class III/IV ± V). Mycophenolate was used for induction in 36%, whereas 34% received cyclophosphamide (CYC); 55% received mycophenolate as maintenance immunosuppression. The rates of complete remission (CR) and partial remission (PR) at 6 months and 12 months, respectively, were 65% and 20% and 78% and 8%. Two patients died (mortality rate 2.1/1000 patient-years), with a standardized mortality ratio of 22.3. Three patients (3.2%) developed end-stage kidney disease (ESKD), and advanced CKD occurred in 5 patients (5.4%). Survival rates without advanced CKD, ESKD, or death were 96.7%, 94.2%, 92.7%, 83.2% and 83.2% at 1 year, 5 years, 10 years, 15 years, and 20 years, respectively. Multivariate analysis revealed that severe kidney failure necessitating dialysis at presentation (adjusted hazard ratio 37.7, 95% confidence interval [CI] 4.0–355.6, P = 0.002), nonresponse (NR) after 12 months of treatment (adjusted hazard ratio 11.2, 95% CI 2.3–54.9, P = 0.003), and multiple nephritis flares (adjusted hazard ratio 2.6, 95% CI 1.1–6.2, P = 0.03) were predictive of advanced CKD, ESKD, or death. Other adverse outcomes included infections (2.9 episodes/100 patient-years), osteopenia (32%), hypertension (17%), short stature (14%), and avascular necrosis (7%). CONCLUSION: The long-term outcomes of cLN appeared to have improved in the present era with effective immunosuppression, cautious drug tapering, and assurance of medication adherence. There is still an unacceptably high prevalence of adverse outcomes.
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spelling pubmed-98319482023-01-12 Long-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis Chan, Eugene Yu-hin Yap, Desmond Yat-hin Wong, Wing-tung Wong, Wilfred Hing-sang Wong, Sze-wa Lin, Kyle Ying-kit Hui, Felix Yan-wai Yee-ming, Jennifer Lam, Sophia Suet-ying Wong, Jennie Kit-yee Lai, Fiona Fung-yee Ho, Tsz-wai Tong, Pak-chiu Lai, Wai-ming Chan, Tak Mao Ma, Alison Lap-tak Kidney Int Rep Clinical Research INTRODUCTION: Long-term data pertaining to childhood-onset lupus nephritis (cLN) remain extremely scarce. METHODS: We conducted a retrospective cohort study of biopsy-proven cLN with onset age <18 years diagnosed from 2001 to 2020 to ascertain the long-term patient and kidney survival rates, and the incidence of advanced chronic kidney disease (CKD) (estimated glomerular filtration rate [eGFR] <60 ml/min per 1.73 m(2)). RESULTS: A total of 92 subjects (78 female; age 13.7 ± 3.3 years; all Chinese) were included, with follow-up duration of 10.3 years (interquartile range, 5.8–15.9). Of these, 83 children (90%) had proliferative lupus nephritis (LN) (Class III/IV ± V). Mycophenolate was used for induction in 36%, whereas 34% received cyclophosphamide (CYC); 55% received mycophenolate as maintenance immunosuppression. The rates of complete remission (CR) and partial remission (PR) at 6 months and 12 months, respectively, were 65% and 20% and 78% and 8%. Two patients died (mortality rate 2.1/1000 patient-years), with a standardized mortality ratio of 22.3. Three patients (3.2%) developed end-stage kidney disease (ESKD), and advanced CKD occurred in 5 patients (5.4%). Survival rates without advanced CKD, ESKD, or death were 96.7%, 94.2%, 92.7%, 83.2% and 83.2% at 1 year, 5 years, 10 years, 15 years, and 20 years, respectively. Multivariate analysis revealed that severe kidney failure necessitating dialysis at presentation (adjusted hazard ratio 37.7, 95% confidence interval [CI] 4.0–355.6, P = 0.002), nonresponse (NR) after 12 months of treatment (adjusted hazard ratio 11.2, 95% CI 2.3–54.9, P = 0.003), and multiple nephritis flares (adjusted hazard ratio 2.6, 95% CI 1.1–6.2, P = 0.03) were predictive of advanced CKD, ESKD, or death. Other adverse outcomes included infections (2.9 episodes/100 patient-years), osteopenia (32%), hypertension (17%), short stature (14%), and avascular necrosis (7%). CONCLUSION: The long-term outcomes of cLN appeared to have improved in the present era with effective immunosuppression, cautious drug tapering, and assurance of medication adherence. There is still an unacceptably high prevalence of adverse outcomes. Elsevier 2022-10-21 /pmc/articles/PMC9831948/ /pubmed/36644360 http://dx.doi.org/10.1016/j.ekir.2022.10.014 Text en © 2022 Published by Elsevier Inc. on behalf of the International Society of Nephrology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Clinical Research
Chan, Eugene Yu-hin
Yap, Desmond Yat-hin
Wong, Wing-tung
Wong, Wilfred Hing-sang
Wong, Sze-wa
Lin, Kyle Ying-kit
Hui, Felix Yan-wai
Yee-ming, Jennifer
Lam, Sophia Suet-ying
Wong, Jennie Kit-yee
Lai, Fiona Fung-yee
Ho, Tsz-wai
Tong, Pak-chiu
Lai, Wai-ming
Chan, Tak Mao
Ma, Alison Lap-tak
Long-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis
title Long-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis
title_full Long-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis
title_fullStr Long-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis
title_full_unstemmed Long-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis
title_short Long-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis
title_sort long-term outcomes of children and adolescents with biopsy-proven childhood-onset lupus nephritis
topic Clinical Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9831948/
https://www.ncbi.nlm.nih.gov/pubmed/36644360
http://dx.doi.org/10.1016/j.ekir.2022.10.014
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