A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report

Abstract Background Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Disease evolution may be severe and refractory to standard treatment including prednisone, azathioprine and cyclophosphamide. Case presentation We pres...

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Autores principales: Margaux Boistault, Mireia Lopez Corbeto, Pierre Quartier, Laura Berbel Arcobé, Ariadna Carsi Durall, Florence A. Aeschlimann
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Publicado: BMC 2021
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Acceso en línea:https://doaj.org/article/0ca2c184ab974bdbbfd6513f2594ed36
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spelling oai:doaj.org-article:0ca2c184ab974bdbbfd6513f2594ed362021-12-05T12:04:36ZA young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report10.1186/s12969-021-00654-71546-0096https://doaj.org/article/0ca2c184ab974bdbbfd6513f2594ed362021-12-01T00:00:00Zhttps://doi.org/10.1186/s12969-021-00654-7https://doaj.org/toc/1546-0096Abstract Background Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Disease evolution may be severe and refractory to standard treatment including prednisone, azathioprine and cyclophosphamide. Case presentation We present the case of a young girl with severe PAN resulting in progressive ischemia and necrosis of fingers and toes. Biological work-up revealed increased acute phase reactants and interleukin-6 levels. She was only partially controlled despite high-dose corticosteroids and cyclophosphamide infusions, and eventually achieved rapid improvement and sustained remission on tocilizumab. Further, we review the current evidence of the interleukin-6-inhibitor tocilizumab for the treatment of PAN. Conclusion Tocilizumab may be an efficient therapeutic option in a subset of treatment-refractory children with PAN.Margaux BoistaultMireia Lopez CorbetoPierre QuartierLaura Berbel ArcobéAriadna Carsi DurallFlorence A. AeschlimannBMCarticlePolyarteritis nodosaChildTocilizumabPediatricsRJ1-570Diseases of the musculoskeletal systemRC925-935ENPediatric Rheumatology Online Journal, Vol 19, Iss 1, Pp 1-6 (2021)
institution DOAJ
collection DOAJ
language EN
topic Polyarteritis nodosa
Child
Tocilizumab
Pediatrics
RJ1-570
Diseases of the musculoskeletal system
RC925-935
spellingShingle Polyarteritis nodosa
Child
Tocilizumab
Pediatrics
RJ1-570
Diseases of the musculoskeletal system
RC925-935
Margaux Boistault
Mireia Lopez Corbeto
Pierre Quartier
Laura Berbel Arcobé
Ariadna Carsi Durall
Florence A. Aeschlimann
A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report
description Abstract Background Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Disease evolution may be severe and refractory to standard treatment including prednisone, azathioprine and cyclophosphamide. Case presentation We present the case of a young girl with severe PAN resulting in progressive ischemia and necrosis of fingers and toes. Biological work-up revealed increased acute phase reactants and interleukin-6 levels. She was only partially controlled despite high-dose corticosteroids and cyclophosphamide infusions, and eventually achieved rapid improvement and sustained remission on tocilizumab. Further, we review the current evidence of the interleukin-6-inhibitor tocilizumab for the treatment of PAN. Conclusion Tocilizumab may be an efficient therapeutic option in a subset of treatment-refractory children with PAN.
format article
author Margaux Boistault
Mireia Lopez Corbeto
Pierre Quartier
Laura Berbel Arcobé
Ariadna Carsi Durall
Florence A. Aeschlimann
author_facet Margaux Boistault
Mireia Lopez Corbeto
Pierre Quartier
Laura Berbel Arcobé
Ariadna Carsi Durall
Florence A. Aeschlimann
author_sort Margaux Boistault
title A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report
title_short A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report
title_full A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report
title_fullStr A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report
title_full_unstemmed A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report
title_sort young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report
publisher BMC
publishDate 2021
url https://doaj.org/article/0ca2c184ab974bdbbfd6513f2594ed36
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