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Article: Long-term outcomes with rituximab as add-on therapy in severe childhood-onset lupus nephritis

TitleLong-term outcomes with rituximab as add-on therapy in severe childhood-onset lupus nephritis
Authors
KeywordsChildhood-onset lupus nephritis
Dialysis
Refractory
Rituximab
Systemic lupus erythematosus
Issue Date26-Jun-2023
PublisherSpringer
Citation
Pediatric Nephrology, 2023 How to Cite?
Abstract

Background Long-term data pertaining to rituximab as add-on therapy in childhood-onset lupus nephritis (cLN) is scarce.

Methods A retrospective cohort study was conducted on all patients with proliferative cLN, diagnosed <= 18 years and between 2005 and 2021, who received rituximab for LN episodes that were life/organ threatening and/or treatment resistant to standard immunosuppression.

Results Fourteen patients with cLN (female, n = 10) were included, with median follow-up period of 6.9 years. LN episodes (class III, n = 1; class IV, n = 11; class IV + V, n = 2) requiring rituximab occurred at 15.6 years (IQR 12.8-17.3), urine protein:creatinine ratio was 8.2 mg/mg (IQR 3.4-10.1) and eGFR was 28 mL/min/1.73 m(2) (IQR 24-69) prior to rituximab treatment. Ten and four patients received rituximab at 1500 mg/m(2) and 750 mg/m(2), which were given at 46.5 days (IQR 19-69) after commencement of standard therapies. Treatment with rituximab resulted in improvements in proteinuria (ps < 0.001), eGFR (ps < 0.01) and serological parameters, including haemoglobin levels, complement 3 levels and anti-dsDNA antibodies, compared with baseline. Rates of complete/partial remission at 6-, 12-and 24-month post-rituximab were 28.6/42.8%, 64.2/21.4% and 69.2/15.3%. All three patients who required acute kidney replacement therapy became dialysis-free after rituximab. Relapse rate following rituximab was 0.11 episodes/patient-year. There was no lethal complication or severe infusion reaction. Hypogammaglobulinaemia was the most frequent complication (45%) but was mostly asymptomatic. Neutropenia and infections were observed in 20% and 25% of treatments. Upon last follow-up, three (21%) and two (14%) patients developed chronic kidney disease (stage 2, n = 2; stage 4; n = 1) and kidney failure, respectively.

Conclusion Add-on rituximab is an effective and safe rescue therapy for cLN patients with life-/organ-threatening manifestations or treatment-resistance.


Persistent Identifierhttp://hdl.handle.net/10722/331937
ISSN
2023 Impact Factor: 2.6
2023 SCImago Journal Rankings: 0.785
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorChan, Eugene Yu-hin-
dc.contributor.authorWong, Sze-wa-
dc.contributor.authorLai, Fiona Fung-yee-
dc.contributor.authorHo, Tsz-wai-
dc.contributor.authorTong, Pak-chiu-
dc.contributor.authorLai, Wai-ming-
dc.contributor.authorMa, Alison Lap-tak-
dc.contributor.authorYap, Desmond Yat-hin-
dc.date.accessioned2023-09-28T04:59:44Z-
dc.date.available2023-09-28T04:59:44Z-
dc.date.issued2023-06-26-
dc.identifier.citationPediatric Nephrology, 2023-
dc.identifier.issn0931-041X-
dc.identifier.urihttp://hdl.handle.net/10722/331937-
dc.description.abstract<p>Background Long-term data pertaining to rituximab as add-on therapy in childhood-onset lupus nephritis (cLN) is scarce.</p><p>Methods A retrospective cohort study was conducted on all patients with proliferative cLN, diagnosed <= 18 years and between 2005 and 2021, who received rituximab for LN episodes that were life/organ threatening and/or treatment resistant to standard immunosuppression.</p><p>Results Fourteen patients with cLN (female, n = 10) were included, with median follow-up period of 6.9 years. LN episodes (class III, n = 1; class IV, n = 11; class IV + V, n = 2) requiring rituximab occurred at 15.6 years (IQR 12.8-17.3), urine protein:creatinine ratio was 8.2 mg/mg (IQR 3.4-10.1) and eGFR was 28 mL/min/1.73 m(2) (IQR 24-69) prior to rituximab treatment. Ten and four patients received rituximab at 1500 mg/m(2) and 750 mg/m(2), which were given at 46.5 days (IQR 19-69) after commencement of standard therapies. Treatment with rituximab resulted in improvements in proteinuria (ps < 0.001), eGFR (ps < 0.01) and serological parameters, including haemoglobin levels, complement 3 levels and anti-dsDNA antibodies, compared with baseline. Rates of complete/partial remission at 6-, 12-and 24-month post-rituximab were 28.6/42.8%, 64.2/21.4% and 69.2/15.3%. All three patients who required acute kidney replacement therapy became dialysis-free after rituximab. Relapse rate following rituximab was 0.11 episodes/patient-year. There was no lethal complication or severe infusion reaction. Hypogammaglobulinaemia was the most frequent complication (45%) but was mostly asymptomatic. Neutropenia and infections were observed in 20% and 25% of treatments. Upon last follow-up, three (21%) and two (14%) patients developed chronic kidney disease (stage 2, n = 2; stage 4; n = 1) and kidney failure, respectively.</p><p>Conclusion Add-on rituximab is an effective and safe rescue therapy for cLN patients with life-/organ-threatening manifestations or treatment-resistance.</p>-
dc.languageeng-
dc.publisherSpringer-
dc.relation.ispartofPediatric Nephrology-
dc.rightsThis work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.-
dc.subjectChildhood-onset lupus nephritis-
dc.subjectDialysis-
dc.subjectRefractory-
dc.subjectRituximab-
dc.subjectSystemic lupus erythematosus-
dc.titleLong-term outcomes with rituximab as add-on therapy in severe childhood-onset lupus nephritis-
dc.typeArticle-
dc.identifier.doi10.1007/s00467-023-06025-6-
dc.identifier.scopuseid_2-s2.0-85162935462-
dc.identifier.eissn1432-198X-
dc.identifier.isiWOS:001020338600003-
dc.identifier.issnl0931-041X-

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