Sistema de Salut de Catalunya
Institut Català de la Salut
[Boistault M, Aeschlimann FA] Department of Pediatric Immunology-Hematology and Rheumatology, Necker University Hospital – Assitance Publique-Hopitaux de Paris, Paris, France. [Lopez Corbeto M] Unitat de Reumatologia Pediàtrica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Quartier P] Department of Pediatric Immunology-Hematology and Rheumatology, Necker University Hospital – Assitance Publique-Hopitaux de Paris, Paris, France. Université de Paris, Imagine Institute, Paris, France. [Berbel Arcobé L] Rheumatology Department, Mútua de Terrassa University Hospital, Terrassa, Spain. [Carsi Durall A] Unitat d’Urgències Pediàtriques, Vall d’Hebron Hospital Universitari, Barcelona, Spain
Vall d'Hebron Barcelona Hospital Campus
2022-05-24T07:11:24Z
2022-05-24T07:11:24Z
2021-12-03
Child; Polyarteritis nodosa; Tocilizumab
Nena; Poliarteritis nodosa; Tocilizumab
Niña; Poliarteritis nodosa; Tocilizumab
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.
Article
Versió publicada
Anglès
Infants; Vasculitis - Tractament; Anticossos monoclonals - Ús terapèutic; DISEASES::Cardiovascular Diseases::Vascular Diseases::Vasculitis::Arteritis::Polyarteritis Nodosa; Other subheadings::Other subheadings::Other subheadings::/drug therapy; CHEMICALS AND DRUGS::Amino Acids, Peptides, and Proteins::Proteins::Blood Proteins::Immunoproteins::Immunoglobulins::Antibodies::Antibodies, Monoclonal::Antibodies, Monoclonal, Humanized; NAMED GROUPS::Persons::Age Groups::Child::Child, Preschool; ENFERMEDADES::enfermedades cardiovasculares::enfermedades vasculares::vasculitis::arteritis::poliarteritis nudosa; Otros calificadores::Otros calificadores::Otros calificadores::/farmacoterapia; COMPUESTOS QUÍMICOS Y DROGAS::aminoácidos, péptidos y proteínas::proteínas::proteínas sanguíneas::inmunoproteínas::inmunoglobulinas::anticuerpos::anticuerpos monoclonales::anticuerpos monoclonales humanizados; DENOMINACIONES DE GRUPOS::personas::Grupos de Edad::niño::niño preescolar
BMC
Pediatric Rheumatology;19
https://doi.org/10.1186/s12969-021-00654-7
Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
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