Background Primary systemic vasculitides (PSV) are very rare in children and consequently, little evidence exists. Evidence-based guidelines are lacking; this is an important and unmet need. The European initiative SHARE (Single Hub and Access point for paediatric Rheumatology in Europe) aims to optimize care for children with rheumatic diseases.

Objectives To provide recommendations for diagnosis and treatment for children with rare forms of PSV.

Methods Recommendations were developed by a consensus process in accordance with the European League Against rheumatism standard operating procedures. An extensive systematic literature was performed and evidence-based or evidence-informed recommendations were extrapolated from the included papers. These were evaluated by a panel of 16 experts via an online survey and subsequently in two consensus meetings, using nominal group technique. Recommendations were accepted when ≥80% of experts agreed.

Results A total of 78 recommendations were accepted in the two consensus meetings. Recommendations pertained to general cross-cutting recommendations for diagnosis and treatment of PSV, as well as disease-specific statements for childhood-onset Polyarteritis Nodosa, Granulomatosis with Polyangiitis, Microscopic Polyangiitis, Eosinophilic Granulomatosis with Polyangiitis, and Takayasu Arteritis.

Conclusions European-wide recommendations for the diagnosis and treatment of rare forms of paediatric PSV have been formulated through an evidence-based consensus process. The SHARE project aims to provide international recommendations and significantly improve the standard of care for children with rheumatic diseases.

Disclosure of Interest None declared