Diagnosis and Treatment of Angiography Positive Medium to Large Vessel Childhood Primary Angiitis of Central Nervous System (p-cPACNS): An International Survey

Frontiers in Pediatrics

Clare E. Pain¹  Angela S. Quan²  Benjamin Rose²  Christian M. Hedrich³  Renate Häfner⁴  Lucia Gerstl⁵  Gabriele Hahn⁶  Martin Smitka⁷  Jürgen Brunner⁸  Fabian Speth⁹ 

[1] Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United Kingdom;Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom;Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom;Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United Kingdom;Deutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany;Division of Paediatric Neurology, Developmental Medicine and Social Paediatrics, Department of Paediatrics, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University Munich, Munich, Germany;Institut und Poliklinik für Radiologie, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany;Klinik und Poliklinik fur Kinder- und Jugendmedizin, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany;Pädiatrische Rheumatologie, Department Kinder- und Jugendheilkunde, Klinisches Ethikkomitee, Medizinische Universität Innsbruck, Innsbruck, Austria;Zentrum für Geburtshilfe, Kinder- und Jugendmedizin, Klinik und Poliklinik für Kinder- und Jugendmedizin, Universitätsklinikum Eppendorf, Hamburg, Germany;

关键词: vasculitis;    CNS;    childhood;    pediatric;    treatment;    diagnosis;    inflammation;    CNS inflammation;   

DOI  :  10.3389/fped.2021.654537

来源: Frontiers

【 摘 要 】

Childhood Primary Angiitis of Central Nervous System (cPACNS) is rare, but can cause significant damage and result in disability or even death. Because of its rarity, the sometimes acute and variable presentation, limited awareness, and the absence of widely accepted diagnostic and therapeutic standards, cPACNS is a diagnostic and therapeutic challenge. Three subcategories of cPACNS exist, including angiography-positive non-progressive p-cPACNS, angiography-positive progressive p-cPACNS which both affects the medium to large vessels, and angiography-negative small vessel sv-cPACNS. Diagnosis and treatment of cPACNS relies on personal experience, expert opinion and case reports/case series. To collect information on diagnostic and therapeutic approaches to transient and progressive cPACNS, a survey was shared among international clinicians (German Society for Pediatric Rheumatology, the Pediatric Rheumatology European Society, the German speaking “Network Pediatric Stroke,” and members of the American College of Rheumatology/CARRA Pediatric Rheumatology list server). Results from this survey will be used to define statements toward a consensus process allowing harmonization of diagnostic and therapeutic approaches and the generation of evidence in a rare condition.

【 授权许可】

CC BY