Background: Lupus nephritis (LN) is more common and aggressive in paediatrics compared to adults. There are several studies in adults showing better outcomes with various immunosuppressive combinations in LN. There is scarcity of such data in paediatric LN.
Objectives: To compare the outcome of childhood-LN with different immunosuppressives in two tertiary Paediatric
Rheumatology Centers, Sir Gangaram Hospital (SGRH) and Great Ormond Street Hospital for Children (GOSH) at 12- months follow-up.
Methods: This is a retrospective study including 101(SGRH=61, GOSH=40) children with biopsy proven class III-IV-VLN, treated between January-2011 to December-2021. Renal outcomes were assessed using the CARRA definitions of substantial response, moderate response and renal flare. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and dose of steroids at onset were calculated. Statistical analysis was performed by T tests and Chi Square static with Yates’ correction.
Results: Median age of onset was 12.6years in SGRH and 13 years in GOSH. M: F ratio was 1:2.8 in SGRH and 1:4 in GOSH. Average SLEDAI at onset was significantly higher in GOSH-24.5 compared to SGRH-17(p<0.05). Intravenous (IV) pulse glucocorticoids (GC) were given in 49/61(80%) and 31/40(78%) in SGRH & GOSH respectively. Average dose of IV GC was significantly higher in GOSH-28.5 vs. SGRH-8.4 mg/kg/day (p<0.05). Average starting dose of oral steroids was higher in SGRH-1 vs. GOSH-0.8mg/kg/day (p<0.05). For Induction (0-6months), SGRH patients received six doses IV cyclophosphamide (CYC, N=18/61), Mycophenolate mofetil (MMF, N=39/61) and Rituximab (RTX, N=4/61) while GOSH patients received RTX+MMF (N=19/40), RTX+ two doses CYC (N=8/40), RTX+Azathioprin (AZA, N=4/40) and MMF (N=9/40). For maintenance (6-12months), SGRH patients received MMF (N=38/61), MMF+Tacrolimus (N=14/61), RTX (N=6/61) and AZA (N=3/61) while GOSH patients received MMF (N=24/40), RTX (N=8/40) and AZA (N=8/40). Overall, there is no significant difference in substantial response (p 0.38), moderate response (p 0.77) and off GC (p 0.85) between the two centers. There is no significant difference between CYC vs. RTX+MMF in substantial response (p 0.88), moderate response (p 0.64), renal flares (p 0.37) and being off GC (p0.79).Similarly, no significant difference between CYC vs. RTX+CYC in substantial response (p 0.92), moderate response (p 0.38), renal flares (p 0.44) and being off GC (p 0.97).
Conclusion: LN outcomes are comparable between the two centers, despite the significant differences in disease severity, dose of GC and choice of immunosuppressives at onset. Larger multicenter studies are needed to look into long-term efficacy and safety of the different immunosuppressive agents in paediatric LN.REFERENCES: NIL.
Special Note:
This abstract has been presented as a Poster Presentation at Pediatric Rheumatology European Society (PReS) 2023 Annual Conference which was held between September 28, 2023 and October 1, 2023 at Rotterdam, The Netherlands.
Acknowledgements: Dr Devang Pandya (Presenting Author) have been awarded by Asia Pacific League of Associations for Rheumatology (APLAR) and PReS EMERGE grants to work as an International Fellow at Great Ormond Street Hospital, London, UK between January 2023 & July 2023. This project was an integral part of this fellowship.
Disclosure of Interests: None declared.