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Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea
Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2021
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8052421/ https://www.ncbi.nlm.nih.gov/pubmed/33863937 http://dx.doi.org/10.1038/s41598-021-87718-6 |
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author | Lee, Jeong-Seon Kim, Joong-Gon Lee, Soyoung |
author_facet | Lee, Jeong-Seon Kim, Joong-Gon Lee, Soyoung |
author_sort | Lee, Jeong-Seon |
collection | PubMed |
description | Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis. |
format | Online Article Text |
id | pubmed-8052421 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Nature Publishing Group UK |
record_format | MEDLINE/PubMed |
spelling | pubmed-80524212021-04-22 Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea Lee, Jeong-Seon Kim, Joong-Gon Lee, Soyoung Sci Rep Article Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis. Nature Publishing Group UK 2021-04-16 /pmc/articles/PMC8052421/ /pubmed/33863937 http://dx.doi.org/10.1038/s41598-021-87718-6 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Article Lee, Jeong-Seon Kim, Joong-Gon Lee, Soyoung Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea |
title | Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea |
title_full | Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea |
title_fullStr | Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea |
title_full_unstemmed | Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea |
title_short | Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea |
title_sort | clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of korea |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8052421/ https://www.ncbi.nlm.nih.gov/pubmed/33863937 http://dx.doi.org/10.1038/s41598-021-87718-6 |
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