Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea

Abstract 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,...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Jeong-Seon Lee, Joong-Gon Kim, Soyoung Lee
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/24371a415882489fb80931bc7f576257
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:24371a415882489fb80931bc7f576257
record_format	dspace
spelling	oai:doaj.org-article:24371a415882489fb80931bc7f5762572021-12-02T14:26:19ZClinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea10.1038/s41598-021-87718-62045-2322https://doaj.org/article/24371a415882489fb80931bc7f5762572021-04-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-87718-6https://doaj.org/toc/2045-2322Abstract 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.Jeong-Seon LeeJoong-Gon KimSoyoung LeeNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-10 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Jeong-Seon Lee Joong-Gon Kim Soyoung Lee Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea
description	Abstract 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	article
author	Jeong-Seon Lee Joong-Gon Kim Soyoung Lee
author_facet	Jeong-Seon Lee Joong-Gon Kim Soyoung Lee
author_sort	Jeong-Seon Lee
title	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_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_sort	clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of korea
publisher	Nature Portfolio
publishDate	2021
url	https://doaj.org/article/24371a415882489fb80931bc7f576257
work_keys_str_mv	AT jeongseonlee clinicalpresentationsandlongtermprognosisofchildhoodonsetpolyarteritisnodosainsinglecentreofkorea AT joonggonkim clinicalpresentationsandlongtermprognosisofchildhoodonsetpolyarteritisnodosainsinglecentreofkorea AT soyounglee clinicalpresentationsandlongtermprognosisofchildhoodonsetpolyarteritisnodosainsinglecentreofkorea
_version_	1718391358356455424

Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea

Ejemplares similares