Seza Ozen1, Stephen D Marks2, Paul Brogan2, Noortje Groot3,4,5, Nienke de Graeff3, Tadej Avcin6, Brigitte Bader-Meunier7, Pavla Dolezalova8, Brian M Feldman9, Isabelle Kone-Paut10, Pekka Lahdenne11, Liza McCann5, Clarissa Pilkington2, Angelo Ravelli12, Annet van Royen3, Yosef Uziel13, Bas Vastert3, Nico Wulffraat3, Sylvia Kamphuis4, Michael W Beresford5,14. 1. Department of Paediatrics, Hacettepe University, Ankara, Turkey. 2. Great Ormond Street Hospital for Children NHS Foundation Trust, University College London Great Ormond Street Institute of Child Health, London, UK. 3. Wilhelmina Children's Hospital, University Medical Center, Utrecht. 4. Sophia Children's Hospital, Erasmus University Medical Centre, Rotterdam, The Netherlands. 5. Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, UK. 6. Department of Paediatric Rheumatology, University Children's Hospital Ljubljana, Ljubljana, Slovenia. 7. Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France. 8. General University Hospital and 1 Faculty of Medicine, Charles University, Prague, Czech Republic. 9. The Hospital for Sick Children, University of Toronto, Toronto, Canada. 10. Department of Paediatric Rheumatology, Bicêtre University Hospital, Paris, France. 11. Hospital for Children and Adolescents, University of Helsinki, Helsinki, Finland. 12. Gaslini Children's Hospital, Genoa, Italy. 13. Meir Medical Centre, Tel Aviv University, Tel Aviv, Israel. 14. Institute of Translational Medicine, University of Liverpool and Alder Hey children's NHS Foundation Trust, Members of Liverpool Health Partners, Liverpool, UK.
Abstract
OBJECTIVES: IgA vasculitis (IgAV, formerly known as Henoch-Schönlein purpura) is the most common cause of systemic vasculitis in childhood. To date, there are no internationally agreed, evidence-based guidelines concerning the appropriate diagnosis and treatment of IgAV in children. Accordingly, treatment regimens differ widely. The European initiative SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) aims to optimize care for children with rheumatic diseases. The aim therefore was to provide internationally agreed consensus recommendations for diagnosis and treatment for children with IgAV. METHODS: Recommendations were developed by a consensus process in accordance with the EULAR standard operating procedures. An extensive systematic literature review was performed, and evidence-based recommendations were extrapolated from the included papers. These were evaluated by a panel of 16 international experts via online surveys and subsequent consensus meeting, using nominal group technique. Recommendations were accepted when ⩾80% of experts agreed. RESULTS: In total, 7 recommendations for diagnosis and 19 for treatment of paediatric IgAV were accepted. Diagnostic recommendations included: appropriate use of skin and renal biopsy, renal work-up and imaging. Treatment recommendations included: the importance of appropriate analgesia and angiotensin-converting enzyme inhibitor use and non-renal indications for CS use, as well as a structured approach to treating IgAV nephritis, including appropriate use of CS and second-line agents in mild, moderate and severe disease along with use of angiotensin-converting enzyme inhibitors and maintenance therapy. CONCLUSION: The SHARE initiative provides international, evidence-based recommendations for the diagnosis and treatment of IgAV that will facilitate improvement and uniformity of care.
OBJECTIVES: IgA vasculitis (IgAV, formerly known as Henoch-Schönlein purpura) is the most common cause of systemic vasculitis in childhood. To date, there are no internationally agreed, evidence-based guidelines concerning the appropriate diagnosis and treatment of IgAV in children. Accordingly, treatment regimens differ widely. The European initiative SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) aims to optimize care for children with rheumatic diseases. The aim therefore was to provide internationally agreed consensus recommendations for diagnosis and treatment for children with IgAV. METHODS: Recommendations were developed by a consensus process in accordance with the EULAR standard operating procedures. An extensive systematic literature review was performed, and evidence-based recommendations were extrapolated from the included papers. These were evaluated by a panel of 16 international experts via online surveys and subsequent consensus meeting, using nominal group technique. Recommendations were accepted when ⩾80% of experts agreed. RESULTS: In total, 7 recommendations for diagnosis and 19 for treatment of paediatric IgAV were accepted. Diagnostic recommendations included: appropriate use of skin and renal biopsy, renal work-up and imaging. Treatment recommendations included: the importance of appropriate analgesia and angiotensin-converting enzyme inhibitor use and non-renal indications for CS use, as well as a structured approach to treating IgAV nephritis, including appropriate use of CS and second-line agents in mild, moderate and severe disease along with use of angiotensin-converting enzyme inhibitors and maintenance therapy. CONCLUSION: The SHARE initiative provides international, evidence-based recommendations for the diagnosis and treatment of IgAV that will facilitate improvement and uniformity of care.