French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides).
Journal
Orphanet journal of rare diseases
ISSN: 1750-1172
Titre abrégé: Orphanet J Rare Dis
Pays: England
ID NLM: 101266602
Informations de publication
Date de publication:
29 12 2020
29 12 2020
Historique:
entrez:
29
12
2020
pubmed:
30
12
2020
medline:
22
6
2021
Statut:
epublish
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.
Identifiants
pubmed: 33372616
doi: 10.1186/s13023-020-01621-3
pii: 10.1186/s13023-020-01621-3
pmc: PMC7771069
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
351Investigateurs
Vincent Audard
(V)
Olivier Aumaitre
(O)
Karine Briot
(K)
Patrice Cacoub
(P)
Pascal Cathebras
(P)
Dominique Chauveau
(D)
Olivier Chosidow
(O)
Laurent Chouchana
(L)
Vincent Cottin
(V)
Divi Cornec
(D)
Eric Daugas
(E)
Elisabeth Diot
(E)
Nicolas Dupin
(N)
Khalil El Karoui
(KE)
Olivier Fain
(O)
Pierre Gobert
(P)
Philippe Guilpain
(P)
Mohamed Hamidou
(M)
Aurélie Hummel
(A)
Marie Jachiet
(M)
Stéphane Jouneau
(S)
Noémie Jourde Chiche
(NJ)
Cédric Landron
(C)
Claire Le Jeunne
(C)
Jean-Christophe Lega
(JC)
Xavier Mariette
(X)
Nathalie Morel
(N)
Christian Pagnoux
(C)
Philippe Remy
(P)
Frédéric Vandergheynst
(F)
Commentaires et corrections
Type : ErratumIn
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