1. French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides)
- Author
-
Terrier, Benjamin, Darbon, Raphaël, Durel, Cécile-Audrey, Hachulla, Eric, Karras, Alexandre, Maillard, Hélène, Papo, Thomas, Puechal, Xavier, Pugnet, Grégory, Quemeneur, Thomas, Samson, Maxime, Taille, Camille, Guillevin, Loïc, Audard, Vincent, Aumaître, Olivier, Briot, Karine, Cacoub, Patrice, Cathebras, Pascal, Chauveau, Dominique, Chosidow, Olivier, Chouchana, Laurent, Cottin, Vincent, Cornec, Divi, Daugas, Eric, Diot, Elisabeth, Dupin, Nicolas, El Karoui, Khalil, Fain, Olivier, Gobert, Pierre, Guilpain, Philippe, Hamidou, Mohamed, Hummel, Aurelie, Jachiet, Marie, Jouneau, Stephane, Jourde-Chiche, Noémie, Landron, Cédric, Le Jeunne, Claire, Lega, Jean-Christophe, Mariette, Xavier, Morel, Nathalie, Pagnoux, Christian, Remy, Philippe, Vandergheynst, Frederic, Service de médecine interne et centre de référence des maladies rares [CHU Cochin], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Association France Vascularites [Paris], Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Néphrologie et Hémodialyse [CHU HEGP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), AP-HP - Hôpital Bichat - Claude Bernard [Paris], CHU Toulouse [Toulouse], Centre hospitalier [Valenciennes, Nord], Service de Médecine Interne (SOC 1 et SOC 2) [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), and Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)
- Subjects
Pediatrics ,medicine.medical_specialty ,lcsh:Medicine ,Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ,Disease ,Churg-Strauss Syndrome ,03 medical and health sciences ,0302 clinical medicine ,Quality of life ,Eosinophilic ,Necrotizing Vasculitis ,medicine ,Humans ,Pharmacology (medical) ,030212 general & internal medicine ,Position Statement ,Genetics (clinical) ,030203 arthritis & rheumatology ,Polyarteritis nodosa ,business.industry ,lcsh:R ,Granulomatosis with Polyangiitis ,General Medicine ,medicine.disease ,3. Good health ,Polyarteritis Nodosa ,[SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics ,Quality of Life ,Granulomatosis with polyangiitis ,business ,Microscopic polyangiitis ,Vasculitis ,[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology - Abstract
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.
- Published
- 2020
- Full Text
- View/download PDF