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Article Dans Une Revue Orphanet Journal of Rare Diseases Année : 2020

French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides)

Vincent Audard
  • Fonction : Collaborateur
Olivier Aumaître
  • Fonction : Collaborateur
Karine Briot
  • Fonction : Collaborateur
Patrice Cacoub
Pascal Cathebras
  • Fonction : Collaborateur
Dominique Chauveau
  • Fonction : Collaborateur
Olivier Chosidow
  • Fonction : Collaborateur
Laurent Chouchana
Vincent Cottin
Divi Cornec
Eric Daugas
  • Fonction : Collaborateur
Elisabeth Diot
  • Fonction : Collaborateur
Nicolas Dupin
  • Fonction : Collaborateur
Khalil El Karoui
  • Fonction : Collaborateur
Olivier Fain
Pierre Gobert
  • Fonction : Collaborateur
Mohamed Hamidou
  • Fonction : Collaborateur
Aurelie Hummel
  • Fonction : Collaborateur
Marie Jachiet
  • Fonction : Collaborateur
Stephane Jouneau
  • Fonction : Collaborateur
Noémie Jourde-Chiche
Cédric Landron
  • Fonction : Collaborateur
Claire Le Jeunne
  • Fonction : Collaborateur
Jean-Christophe Lega
Xavier Mariette
Nathalie Morel
Christian Pagnoux
  • Fonction : Collaborateur
Philippe Remy
  • Fonction : Collaborateur
Frederic Vandergheynst
  • Fonction : Collaborateur

Résumé

Systemic necrotizing vasculitis comprises a group of diseases resembling polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA): granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis. The definitive diagnosis is made in cooperation with a reference center for autoimmune diseases and rare systemic diseases or a competency center. The management goals are: to obtain remission and, in the long term, healing; to reduce the risk of relapses; to limit and reduce the sequelae linked to the disease; to limit the side effects and the sequelae linked to the treatments; to improve or at least maintain the best possible quality of life; and to maintain socio-professional integration and/or allow a rapid return to school and/or professional activity. Information and therapeutic education of the patients and those around them are an integral part of the care. All health professionals and patients should be informed of the existence of patient associations. The treatment of vasculitis is based on variable combinations of glucocorticoids and immunosuppressants, chosen and adapted according to the disease concerned, the severity and/or extent of the disease, and the underlying factors (age, kidney function, etc.). Follow-up clinical and paraclinical examinations must be carried out regularly to clarify the progression of the disease, detect and manage treatment failures and possible relapses early on, and limit sequelae and complications (early then late) related to the disease or treatment. A distinction is made between the induction therapy, lasting approximately 3-6 months and aimed at putting the disease into remission, and the maintenance treatment, lasting 12-48 months, or even longer. The role of the increase or testing positive again for ANCA as a predictor of a relapse, which has long been controversial, now seems to have greater consensus: Anti-myeloperoxidase ANCAs are less often associated with a relapse of vasculitis than anti-PR3 ANCA.
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hal-03170911 , version 1 (16-03-2021)

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Benjamin Terrier, Raphaël Darbon, Cécile-Audrey Durel, Eric Hachulla, Alexandre Karras, et al.. French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides). Orphanet Journal of Rare Diseases, 2020, 15 (S2), pp.351. ⟨10.1186/s13023-020-01621-3⟩. ⟨hal-03170911⟩
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