Autoinflammatory & rare systemic diseases

International multidisciplinary consensus on the definition and clinical approach for monogenic inflammatory immune dysregulation disorders

Pediatr Rheumatol Online JPublished Consensus statement

Methodological detailssystematic literature review, pre-Delphi electronic survey, modified online Delphi process, international multidisciplinary expert panel, Consensus defined as ≥ 80% agreement; two Delphi rounds, Literature review updated through December 31, 2024, Pre-Delphi survey completed by 95 international adult and pediatric immunologists and rheumatologists
  • Systematic literature review
  • pre-Delphi electronic survey
  • Modified online Delphi process
  • International multidisciplinary expert panel
  • Consensus defined as ≥ 80% agreement; two Delphi rounds
  • Literature review updated through December 31, 2024
  • Pre-Delphi survey completed by 95 international adult and pediatric immunologists and rheumatologists

Scope

As issued by the authors

Definition and clinical approach of Monogenic Inflammatory Immune Dysregulation Disorders (MIIDDs) in five domains: definitions and conceptual framework; diagnostic and monitoring considerations; treatment and therapeutic strategies; multidisciplinary and collaborative care; patient education and support

Original publication

International multidisciplinary consensus on the definition and clinical approach for monogenic inflammatory immune dysregulation disorders. Pediatr Rheumatol Online J. Published 14 May 2025. doi:10.1186/s12969-025-01109-z

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