Title :

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

Author(s) :

Terrier, Benjamin [Auteur]
Hôpital Cochin [AP-HP]
Darbon, Raphael [Auteur]
Durel, Cecile-Audrey [Auteur]
Hospices Civils de Lyon [HCL]
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Karras, Alexandre [Auteur]
Service de Néphrologie et Hémodialyse [CHU HEGP]
Maillard, Hélène [Auteur]
Centre Hospitalier Régional Universitaire [CHU Lille] [CHRU Lille]
Papo, Thomas [Auteur]
AP-HP - Hôpital Bichat - Claude Bernard [Paris]
Puechal, Xavier [Auteur]
Hôpital Cochin [AP-HP]
Pugnet, Gregory [Auteur]
Centre Hospitalier Universitaire de Toulouse [CHU Toulouse]
Quemeneur, Thomas [Auteur]
Centre hospitalier [Valenciennes, Nord]
Samson, Maxime [Auteur]
CHU Dijon
Taille, Camille [Auteur]
AP-HP - Hôpital Bichat - Claude Bernard [Paris]
Guillevin, Loic [Auteur]
Hôpital Cochin [AP-HP]

Journal title :

Orphanet Journal of Rare Diseases

Abbreviated title :

Orphanet J Rare Dis

Volume number :

15

Pages :

351

Publication date :

2020-12-29

ISSN :

1750-1172

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Systemic necrotizing vasculitis comprises a group of diseases resembling polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA): granulomatosis with polyangiitis, eosinophilic granulomatosis ...
Show more >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.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

CHU Lille
Inserm
Université de Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2021-07-06T12:44:53Z
2024-03-08T16:43:22Z