MON-308 MANAGEMENT OF HENOCH-SCHONLEIN PURPURA NEPHRITIS IN AUSTRALIA AND NEW ZEALAND: DOES IT DIFFER FROM KIDNEY DISEASE IMPROVING GLOBAL OUTCOMES GUIDELINES?

Abstract

Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children, with renal involvement occurring in 40-60% of paediatric patients within 4 to 6 weeks of the initial presentation. Majority of paediatric patients with HSP nephritis (HSPN) have mild disease, however a small proportion of patients present with renal impairment, nephrotic syndrome, or both. Kidney Disease Improving Global Outcomes (KDIGO) guidelines assist with management of different severities of HSPN but with low level of evidence. We aimed to evaluate the variance in current practice in management of paediatric HSPN by paediatric nephrologists in Australia and New Zealand. We designed an electronic survey consisting of various clinical scenarios of different severities in paediatric patients with HSPN. All of the questions were in multiple-choice format; majority allowing for participants to provide multiple management choices if required. The link to the survey was e-mailed to all members (trainees and consultants) of the Australian and New Zealand Paediatric Nephrology Association (ANZPNA). Survey was sent to 55 members of ANZPA. The responses were collated and analysed. The response rate was 30%, majority of the respondents being Staff Specialists of more than 10 years of experience. Majority of the respondents would commence treatment for mild HSPN with angiotensin converting enzyme inhibitors (ACEi), which is consistent with KDIGO guideline. Prednisolone was added in conjunction with ACEi for HSPN International Study of Kidney Diseases in Children (ISKDC) classification II and above. More complex HSPN including macroscopic haematuria and nephrotic-range proteinuria highlighted variability in practice amongst the ANZPNA group. For ISDKC classification IIIb and more, a non-steroid based immunosuppressive therapy was added to other therapies, but there was no consistency in treatment of choice. Rapidly progressive glomerulonephritis (RPGN) scenario was usually treated with IV methylprednisolone and cyclophosphamide with occasional use of plasma exchange. The majority of Australia and New Zealand Paediatric Nephrologists follow the KDIGO recommendations for treatment of mild HSPN. There is a wide variation in practice for moderate-to-severe HSPN with no consistency in choice of non-steroid immunosuppression, based on clinical presentation or histological findings except for RPGN. The wide variation in clinical practice highlights the lack of current evidence-based recommendations for HSPN treatment.