File Download

There are no files associated with this item.

  Links for fulltext
     (May Require Subscription)
Supplementary

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

TitleLong-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis
Authors
Keywordschildhood-onset
chronic kidney disease
dialysis
lupus nephritis
mortality
systemic lupus erythematosus
Issue Date21-Oct-2022
PublisherElsevier
Citation
Kidney International Reports, 2023, v. 8, n. 1, p. 141-150 How to Cite?
Abstract

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 m2).

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. © 2022 International Society of Nephrology


Persistent Identifierhttp://hdl.handle.net/10722/329169
ISSN
2023 Impact Factor: 5.7
2023 SCImago Journal Rankings: 1.377
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorChan, EYH-
dc.contributor.authorYap, DYH-
dc.contributor.authorWong, WT-
dc.contributor.authorWong, WHS-
dc.contributor.authorWong, SW-
dc.contributor.authorLin, KYK-
dc.contributor.authorHui, FYW-
dc.contributor.authorYee-ming, J-
dc.contributor.authorLam, SSY-
dc.contributor.authorWong, JKY-
dc.contributor.authorLai, FFY-
dc.contributor.authorHo, TW-
dc.contributor.authorTong, PC-
dc.contributor.authorLai, WM-
dc.contributor.authorChan, TM-
dc.contributor.authorMa, ALT-
dc.date.accessioned2023-08-05T07:55:48Z-
dc.date.available2023-08-05T07:55:48Z-
dc.date.issued2022-10-21-
dc.identifier.citationKidney International Reports, 2023, v. 8, n. 1, p. 141-150-
dc.identifier.issn2468-0249-
dc.identifier.urihttp://hdl.handle.net/10722/329169-
dc.description.abstract<p>Introduction: Long-term data pertaining to childhood-onset lupus nephritis (cLN) remain extremely scarce. <br></p><p>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<sup>2</sup>). <br></p><p>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%). <br></p><p>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. © 2022 International Society of Nephrology</p>-
dc.languageeng-
dc.publisherElsevier-
dc.relation.ispartofKidney International Reports-
dc.rightsThis work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.-
dc.subjectchildhood-onset-
dc.subjectchronic kidney disease-
dc.subjectdialysis-
dc.subjectlupus nephritis-
dc.subjectmortality-
dc.subjectsystemic lupus erythematosus-
dc.titleLong-Term Outcomes of Children and Adolescents With Biopsy-Proven Childhood-Onset Lupus Nephritis-
dc.typeArticle-
dc.identifier.doi10.1016/j.ekir.2022.10.014-
dc.identifier.scopuseid_2-s2.0-85141961923-
dc.identifier.volume8-
dc.identifier.issue1-
dc.identifier.spage141-
dc.identifier.epage150-
dc.identifier.eissn2468-0249-
dc.identifier.isiWOS:000917448200001-
dc.identifier.issnl2468-0249-

Export via OAI-PMH Interface in XML Formats


OR


Export to Other Non-XML Formats