European consensus-based recommendations for diagnosis and treatment of immunoglobulin A vasculitis—the SHARE initiative

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.

Predicting renal outcomes in immunoglobulin A vasculitis nephritis; from ISKDC classification to Oxford MEST-C classification

“…IgAV is a systemic vasculitis (leukocytoclastic vasculitis) affecting the small vessels and is characterized by the deposition of IgA-containing immune complexes, particularly in the kidneys, skin and other organs. IgAVN and IgAN are believed to be related diseases, since both possibly share a related immunopathogenic mechanism which engages abnormally glycosylated IgA1 (12,13). IgAVN tends to display itself as an acute proliferative glomerulonephritis, while IgAN tends to have an indolent course.…”

Ab0998 severe Iga Vasculitis: Cyclophosphamide in Question

“…(1) No specific recommendations are available for cerebral vasculitis of IgAV. Objectives: To describe disease course and treatment response in 2 patients with severe IgAV with CNS and renal involvement.…”

Nefrite na púrpura Henoch-Schönlein: fatores de risco iniciais e evolução em um centro terciário da América Latina

“…A Púrpura de Henoch-Schönlein (PHS), atualmente também denominada vasculite por imunoglobulina A (IgA) 1 , apresentou sua nomenclatura revisada após a Conferência do Consenso Internacional de Chapel Hill de 2012, que considerou a fisiopatologia principalmente envolvida na doença que é o depósito predominante de IgA1 deficiente em galactose na parede dos vasos acometidos [1][2][3][4][5][6] .…”

“…A PHS é a vasculite primária mais frequente em crianças e adolescentes, com incidência de 3 a 30 casos por 100.000 crianças e adolescentes por ano [2][3][4][5][6] . É caracterizada por inflamação de vasos de pequeno calibre (arteríolas, vênulas e capilares) e envolve tipicamente a pele, articulações, trato gastrointestinal (GI) e rins [1][2][3] . A média de idade de início da doença é 6 anos, sendo que 90% dos casos ocorre em crianças menores de 10 anos, com predomínio no sexo masculino em relação ao feminino (relação 1,5:1).…”

“…Pode ainda ocorrer síndrome nefrítica, síndrome nefrótica, hipertensão ou insuficiência renal. A morbidade é alta se houver na fase aguda da doença glomerulonefrite rapidamente progressiva (com crescentes à biópsia renal), assim como, alterações renais persistentes na evolução 3 . Posto isto, a nefrite da PHS (NPHS) é o principal determinante prognóstico a longo prazo da doença, podendo evoluir com fibrose tubulointersticial e culminando em falência renal [8][9][10] .…”