MANAGEMENT OF PAEDIATRIC IGA VASCULITIS WITH NEPHRITIS: A SINGLE-CENTRE EXPERIENCE
Dr THOMAS MILLER1, Dr AMELIA LE PAGE2,3, Professor A. RICHARD KITCHING2,3,4,5, Dr LILIAN JOHNSTONE2,3
1Nephrology Department, Women’s And Children’s Hospital, SA Health, Australia, 2Department of Paediatric Nephrology, Monash Children’s Hospital, Monash Health, Australia, 3Department of Paediatrics, Monash University, Australia, 4Department of Nephrology, Monash Health, Australia, 5Department of Medicine, Monash University, Australia
Aim: To review the management of childhood IgA vasculitis with nephritis (IgAVN) at Monash Children’s Hospital.
Background: IgA vasculitis is the most common form of childhood vasculitis, with nephritis creating a long-term health risk. Guidelines are based on retrospective data and expert opinion, with variation in practice.
Methods: Children diagnosed with IgAVN from January 2012 to June 2023 were identified via retrospective chart review. Demographic and clinical data collected included presenting features, laboratory data, biopsy details, treatment and outcomes.
Results: During the study period, 40 children with IgAVN had sufficient follow-up data available. Median age at presentation was 6.2 years (IQR 4.0-10.6), with 58% male patients. Renal disease included haematuria (38 patients, macroscopic in 12), proteinuria (38 patients, nephrotic-range in 23), renal impairment (12 patients), and hypertension (10 patients). Presentations included nephrotic syndrome (n=1), nephritic syndrome (n=5), and persistent proteinuria (n=17). No patients had rapidly progressive glomerulonephritis.
Renal biopsy was performed in 23 patients, mainly for nephrotic-range proteinuria (n=14). Most patients (n=12) demonstrated International Study of Kidney Disease in Children grade III disease. Oral corticosteroids were used in 21 patients for renal disease, with 5 receiving intravenous methylprednisolone. Other immunosuppressants used included azathioprine (n=4), mycophenolate mofetil (n=2), ciclosporin (n=5), and tacrolimus (n=2). Renin-angiotensin-system blockade was used in 23 patients.
Median follow-up length was 20.6 months (IQR 10.3-36.7). Complete remission was present in 28 patients, and partial remission was noted in 6 patients. Nonresponsive renal manifestations included proteinuria (6 patients) and reduced eGFR (4 patients).
Conclusions: Indications for renal biopsy largely conformed with current international guidelines, while therapy was applied less consistently. Chronic kidney disease (proteinuria or renal impairment) persisted in 23% of patients.
Biography:
Thomas is a second year paediatric nephrology advance trainee at the Women’s and Children’s Hospital. He completed his first year of advanced training at Monash Children’s Hospital.