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Long-term outcomes with rituximab as add-on therapy in severe childhood-onset lupus nephritis.
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- Author(s): Chan, Eugene Yu-hin; Wong, Sze-wa; Lai, Fiona Fung-yee; Ho, Tsz-wai; Tong, Pak-chiu; Lai, Wai-ming; Ma, Alison Lap-tak; Yap, Desmond Yat-hin
- Source:
Pediatric Nephrology; Dec2023, Vol. 38 Issue 12, p4001-4011, 11p- Subject Terms:
RITUXIMAB; DRUG efficacy; PROTEINS; GLOMERULAR filtration rate; AUTOANTIBODIES; CHRONIC kidney failure; LUPUS nephritis; HEMOGLOBINS; COMPLEMENT (Immunology); DNA; RETROSPECTIVE studies; IMMUNOSUPPRESSION; NEUTROPENIA; SEVERITY of illness index; AGE factors in disease; PROTEINURIA; DESCRIPTIVE statistics; AGAMMAGLOBULINEMIA; HEMODIALYSIS; SYSTEMIC lupus erythematosus; LONGITUDINAL method; CREATININE; PATIENT safety; DISEASE risk factors; EVALUATION; DISEASE complications; CHILDREN; ADOLESCENCE - Source:
- Additional Information
- 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 m2 (IQR 24–69) prior to rituximab treatment. Ten and four patients received rituximab at 1500 mg/m2 and 750 mg/m2, 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. [ABSTRACT FROM AUTHOR]
- Abstract: Copyright of Pediatric Nephrology is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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