Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey
Childhood primary angiitis of the Central Nervous System (cPACNS) is a rare autoimmune and inflammatory disease. It can result in significant neuronal damage, neurodevelopmental delay and potentially death. Childhood PACNS is divided into subcategories: angiography-positive p-cPACNS that affects med...
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| Format: | Article |
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Frontiers Media S.A.
2021-10-01
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| Series: | Frontiers in Pediatrics |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fped.2021.756612/full |
| _version_ | 1818694196278591488 |
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| author | Paul Keenan Jürgen Brunner Angela S. Quan Martin Smitka Gabriele Hahn Clare E. Pain Renate Häfner Fabian Speth Lucia Gerstl Christian M. Hedrich Christian M. Hedrich |
| author_facet | Paul Keenan Jürgen Brunner Angela S. Quan Martin Smitka Gabriele Hahn Clare E. Pain Renate Häfner Fabian Speth Lucia Gerstl Christian M. Hedrich Christian M. Hedrich |
| author_sort | Paul Keenan |
| collection | DOAJ |
| description | Childhood primary angiitis of the Central Nervous System (cPACNS) is a rare autoimmune and inflammatory disease. It can result in significant neuronal damage, neurodevelopmental delay and potentially death. Childhood PACNS is divided into subcategories: angiography-positive p-cPACNS that affects medium and large vessels, and angiography-negative small vessel sv-cPACNS. Due to its rarity, variable clinical representation, and the lack of a diagnostic criteria and therapeutic plans, diagnosis and treatment of cPACNS is challenging and approaches vary. This survey collected information on diagnostic and therapeutic approaches to sv-PACNS. It was shared with international clinician networks, including the German Society for Paediatric Rheumatology, the Paediatric Rheumatology European Society, the “Network Paediatric Stroke,” and members of the American College of Rheumatology/CARRA Paediatric Rheumatology list server. This project has shown consensus in numerous diagnostic and therapeutic treatment approaches, highlighting key areas which will be utilised to develop statements in the use of expert consensus meetings to standardise diagnostic and therapeutic approaches in this rare inflammatory disease. |
| first_indexed | 2024-12-17T13:25:44Z |
| format | Article |
| id | doaj.art-7b0ef60d9f0a4d16a26d5c147efb62e7 |
| institution | Directory Open Access Journal |
| issn | 2296-2360 |
| language | English |
| last_indexed | 2024-12-17T13:25:44Z |
| publishDate | 2021-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Pediatrics |
| spelling | doaj.art-7b0ef60d9f0a4d16a26d5c147efb62e72022-12-21T21:46:44ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602021-10-01910.3389/fped.2021.756612756612Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International SurveyPaul Keenan0Jürgen Brunner1Angela S. Quan2Martin Smitka3Gabriele Hahn4Clare E. Pain5Renate Häfner6Fabian Speth7Lucia Gerstl8Christian M. Hedrich9Christian M. Hedrich10Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United KingdomPädiatrische Rheumatologie, Department Kinder- und Jugendheilkunde, Medizinische Universität Innsbruck, Innsbruck, AustriaDepartment of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United KingdomKlinik und Poliklinik fur Kinder- und Jugendmedizin, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyInstitut und Poliklinik für Radiologie, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, GermanyDepartment of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United KingdomDeutsches Zentrum für Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, GermanyZentrum für Geburtshilfe, Kinder- und Jugendmedizin, Klinik und Poliklinik für Kinder- und Jugendmedizin, Universitätsklinikum Eppendorf, Hamburg, GermanyDivision of Paediatric Neurology, Developmental Medicine and Social Paediatrics, Department of Paediatrics, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University Munich, Munich, GermanyDepartment of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United KingdomDepartment of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United KingdomChildhood primary angiitis of the Central Nervous System (cPACNS) is a rare autoimmune and inflammatory disease. It can result in significant neuronal damage, neurodevelopmental delay and potentially death. Childhood PACNS is divided into subcategories: angiography-positive p-cPACNS that affects medium and large vessels, and angiography-negative small vessel sv-cPACNS. Due to its rarity, variable clinical representation, and the lack of a diagnostic criteria and therapeutic plans, diagnosis and treatment of cPACNS is challenging and approaches vary. This survey collected information on diagnostic and therapeutic approaches to sv-PACNS. It was shared with international clinician networks, including the German Society for Paediatric Rheumatology, the Paediatric Rheumatology European Society, the “Network Paediatric Stroke,” and members of the American College of Rheumatology/CARRA Paediatric Rheumatology list server. This project has shown consensus in numerous diagnostic and therapeutic treatment approaches, highlighting key areas which will be utilised to develop statements in the use of expert consensus meetings to standardise diagnostic and therapeutic approaches in this rare inflammatory disease.https://www.frontiersin.org/articles/10.3389/fped.2021.756612/fullvasculitisCNSchildhoodpediatrictreatmentdiagnosis |
| spellingShingle | Paul Keenan Jürgen Brunner Angela S. Quan Martin Smitka Gabriele Hahn Clare E. Pain Renate Häfner Fabian Speth Lucia Gerstl Christian M. Hedrich Christian M. Hedrich Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey Frontiers in Pediatrics vasculitis CNS childhood pediatric treatment diagnosis |
| title | Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey |
| title_full | Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey |
| title_fullStr | Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey |
| title_full_unstemmed | Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey |
| title_short | Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey |
| title_sort | diagnosis and treatment of small vessel childhood primary angiitis of the central nervous system sv cpacns an international survey |
| topic | vasculitis CNS childhood pediatric treatment diagnosis |
| url | https://www.frontiersin.org/articles/10.3389/fped.2021.756612/full |
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