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

被引：53

作者：

Terrier, Benjamin ^{[1
]}

Darbon, Raphael ^{[2
]}

Durel, Cecile-Audrey ^{[3
]}

Hachulla, Eric ^{[4
]}

Karras, Alexandre ^{[5
]}

Maillard, Helene ^{[4
]}

Papo, Thomas ^{[6
]}

Puechal, Xavier ^{[1
]}

Pugnet, Gregory ^{[7
]}

Quemeneur, Thomas ^{[8
]}

Samson, Maxime ^{[9
]}

Taille, Camille ^{[10
]}

Guillevin, Loic ^{[1
]}

Audard, Vincent ^{[11
]}

Aumaitre, Olivier ^{[12
]}

Briot, Karine ^{[13
]}

Cacoub, Patrice ^{[14
]}

Cathebras, Pascal ^{[15
]}

Chauveau, Dominique ^{[16
]}

Chosidow, Olivier ^{[17
]}

Chouchana, Laurent ^{[18
]}

Cottin, Vincent ^{[19
]}

Cornec, Divi ^{[20
]}

Daugas, Eric ^{[21
]}

Diot, Elisabeth ^{[22
]}

Dupin, Nicolas ^{[23
]}

El Karoui, Khalil ^{[24
]}

Fain, Olivier ^{[25
]}

Gobert, Pierre ^{[26
]}

Guilpain, Philippe ^{[27
]}

Hamidou, Mohamed ^{[28
]}

Hummel, Aurelie ^{[29
]}

Jachiet, Marie ^{[30
]}

Jouneau, Stephane ^{[31
]}

Chiche, Noemie Jourde ^{[32
]}

Landron, Cedric ^{[33
]}

Le Jeunne, Claire ^{[34
]}

Lega, Jean-Christophe ^{[35
]}

Mariette, Xavier ^{[36
]}

Morel, Nathalie ^{[38
]}

Pagnoux, Christian ^{[39
]}

Remy, Philippe ^{[11
]}

Vandergheynst, Frederic ^{[40
]}

机构：

[1] CHU Cochin, AP HP, Internal Med, Paris, France

[2] French Vasculitis Assoc, Paris, France

[3] CHU Lyon, Internal Med, Lyon, France

[4] CHU Lille, Internal Med, Lille, France

[5] HEGP, AP HP, Nephrol, Paris, France

[6] CHU Bichat, AP HP, Internal Med, Paris, France

[7] CHU Toulouse, Internal Med, Toulouse, France

[8] CH Valenciennes, Internal Med, Valenciennes, France

[9] CHU Dijon, Internal Med, Dijon, France

[10] CHU Bichat, AP HP, Pulmonol, Paris, France

[11] CHU Henri Mondor, AP HP, Nephrol, Henri Mondor, France

[12] CHU Clermont, Internal Med, Clermont Ferrand, France

[13] CHU Cochin, AP HP, Rheumatol, Cochin, India

[14] CHU Pitie Salpetriere, AP HP, Internal Med, Pitie Salpetriere, France

[15] CHU St Etienne, Internal Med, St Etienne, France

[16] CHU Toulouse, Nephrol, Toulouse, France

[17] CHU Henri Mondor, Dermatol, Paris, France

[18] CHU Cochin, Dermatol, Paris, France

[19] CHU Lyon, Pulmonol, Lyon, France

[20] CHU Brest, Rheumatol, Brest, France

[21] CHU Bichat, AP HP, Nephrol, Bichat, France

[22] CHU Tours, Internal Med, Tours, France

[23] CHU Cochin, AP HP, Dermatol, Cochin, India

[24] CHU Henri Mondor, AP HP, Nephrol, Paris, France

[25] CHU St Antoine, AP HP, Internal Med, St Antoine, France

[26] Rhone Durance Clin, Nephrol, Avignon, France

[27] CHU Montpellier, Internal Med, Montpellier, France

[28] CHU Nantes, Internal Med, Nantes, France

[29] CHU Necker, AP HP, Nephrol, Necker, France

[30] CHU St Louis, AP HP, Dermatol, St Louis, France

[31] CHU Rennes, Pulmonol, Rennes, France

[32] CHU Marseille, Nephrol, Marseille, France

[33] CHU Poitiers, Internal Med, Poitiers, France

[34] CHU Cochin, AP HP, Internal Med, Cochin, India

[35] CHU Lyon, Internal Med, Lyon, France

[36] CHU Kremlin Bicetre, AP HP, Rheumatol, Le Kremlin Bicetre, France

[37] Mt Sinai Hosp, Toronto, ON, Canada

[38] CHU Cochin, AP HP, Gen Med, Cochin, Belgium

[39] Mt Sinai Hosp, Toronto, ON, Canada

[40] CHU Brussels, Internal Med, Brussels, Belgium

来源：

ORPHANET JOURNAL OF RARE DISEASES | 2020年 / 15卷 / SUPPL 2期

关键词：

CHURG-STRAUSS-SYNDROME; RHEUMATOLOGY; 1990; CRITERIA; HEPATITIS-B-VIRUS; TERM-FOLLOW-UP; POOR-PROGNOSIS FACTORS; ANTIBODY-ASSOCIATED VASCULITIS; TRIAL COMPARING GLUCOCORTICOIDS; DAILY ORAL CYCLOPHOSPHAMIDE; PULMONARY-RENAL SYNDROME; WEGENERS-GRANULOMATOSIS;

D O I：

10.1186/s13023-020-01621-3

中图分类号：

Q3 [遗传学];

学科分类号：

071007 ; 090102 ;

摘要：

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.

引用

页数：44

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