ERN-RITA/PReS recommendations for the diagnosis, classification, monitoring, and treatment of childhood idiopathic (primary) angiitis of the central nervous system
Methodological detailssystematic literature review, modified Delphi survey and face-to-face consensus conference, Adapting the European Alliance of Associations for Rheumatology standardised operating procedures, systematic literature review across 4 databases, graded per the Oxford Centre for Evidence-Based Medicine hierarchy, 2-day face-to-face consensus conference, ≥70% agreement required
- Systematic literature review, modified Delphi survey and face-to-face consensus conference
- Adapting the European Alliance of Associations for Rheumatology standardised operating procedures
- Systematic literature review across 4 databases
- Graded per the Oxford Centre for Evidence-Based Medicine hierarchy
- 2-day face-to-face consensus conference
- ≥70% agreement required
Recommendations
As issued by the authors
an 8-domain nomenclature system replacing the 'primary/secondary' dichotomy with 'idiopathic/disease-associated' terminology
a paediatric-specific vessel wall imaging interpretive principle
a prospective risk-stratification model for disease progression
genome-wide sequencing as the preferred genetic testing approach
formal definitions of remission, relapse, and refractory disease
Original publication
ERN-RITA/PReS recommendations for the diagnosis, classification, monitoring, and treatment of childhood idiopathic (primary) angiitis of the central nervous system. Ann Rheum Dis. Published 12 November 2026. doi:10.
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