L BUTLER1,2, A LE PAGE1,2, A KITCHING1,3,4, L JOHNSTONE1,2
1Department of Nephrology, Monash Children’s Hospital, Clayton, Australia, 2Department of Paediatrics, Monash University, Clayton, Australia, 3Department of Medicine, Monash University, Clayton, Australia, 4Department of Nephrology, Monash Health, Clayton, Australia
Background: Lupus nephritis (LN) is a poor prognostic marker in children with Systemic Lupus Erythematosus (SLE). Treatment strategies are based on adult guidelines, involving multiple immunosuppressive agents.
Aim: In children with LN at Monash Children’s Hospital (MCH), to review induction treatment and immunosuppressive agents used compared to international guidelines, and to describe patient outcomes.
Methods: Retrospective chart review identified children diagnosed with SLE and LN at MCH from January 2002-June 2020. Demographic and clinical data were collected, including relapses or progression of LN and modalities of treatment.
Results: Over the study period, 17 children with LN were treated at MCH. Data from 16 patients was available. 68.7% were female, with median age at diagnosis 12.5 years (range 8–17). 15 patients had LN at presentation: all had proteinuria (nephrotic range in 7), 14 had haematuria and no patient had renal impairment. All had systemic features, most commonly rash, fever and arthritis. 15 of 16 patients underwent renal biopsy, with proliferative LN in 66.6%. All patients received oral hydroxychloroquine and glucocorticoids. 81.2% received intravenous methylprednisolone and 75% commenced mycophenolate mofetil at induction. 8 of 15 had at least one further kidney biopsy, with a change in LN class observed in five patients. Three patients had progressive LN- requiring treatment with cyclophosphamide, rituximab and ultimately dialysis (median age 15, median time to dialysis 5 years). Excluding the 3 patients with end stage kidney disease, the median eGFR at last follow-up (median 2.25 years) was 108ml/min/1.73m2 (2 patients had an eGFR <90ml/min/1.73m2).
Conclusions: Induction treatment of paediatric patients with LN at MCH aligned with recommendations of current international guidelines. End stage kidney disease developed in 18.7% of patients.
Louisa is a second-year paediatric nephrology advanced trainee at Monash Children’s Hospital, having completed her first year of training at the Royal Children’s Hospital.