Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey

Keenan, Paul, Brunner, Juergen, Quan, Angela S, Smitka, Martin, Hahn, Gabriele, Pain, Clare E, Haefner, Renate, Speth, Fabian, Gerstl, Lucia and Hedrich, Christian M ORCID: 0000-0002-1295-6179
(2021) Diagnosis and Treatment of Small Vessel Childhood Primary Angiitis of the Central Nervous System (sv-cPACNS): An International Survey. FRONTIERS IN PEDIATRICS, 9. 756612-.

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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.

Item Type: Article
Uncontrolled Keywords: vasculitis, CNS, childhood, pediatric, treatment, diagnosis, inflammation, CNS inflammation
Divisions: Faculty of Health and Life Sciences
Faculty of Health and Life Sciences > Institute of Life Courses and Medical Sciences
Depositing User: Symplectic Admin
Date Deposited: 08 Dec 2021 10:53
Last Modified: 18 Jan 2023 21:23
DOI: 10.3389/fped.2021.756612
Open Access URL: https://doi.org/10.3389/fped.2021.756612
Related URLs:
URI: https://livrepository.liverpool.ac.uk/id/eprint/3144942