French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides)
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...
| Main Authors: | , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2020-12-01
|
| Series: | Orphanet Journal of Rare Diseases |
| Online Access: | https://doi.org/10.1186/s13023-020-01621-3 |
| _version_ | 1818647655075545088 |
|---|---|
| author | Benjamin Terrier Raphaël Darbon Cécile-Audrey Durel Eric Hachulla Alexandre Karras Hélène Maillard Thomas Papo Xavier Puechal Grégory Pugnet Thomas Quemeneur Maxime Samson Camille Taille Loïc Guillevin Collaborators |
| author_facet | Benjamin Terrier Raphaël Darbon Cécile-Audrey Durel Eric Hachulla Alexandre Karras Hélène Maillard Thomas Papo Xavier Puechal Grégory Pugnet Thomas Quemeneur Maxime Samson Camille Taille Loïc Guillevin Collaborators |
| author_sort | Benjamin Terrier |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-17T01:05:59Z |
| format | Article |
| id | doaj.art-07da736fe4594cf69bc4c55e27ac8203 |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-12-17T01:05:59Z |
| publishDate | 2020-12-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-07da736fe4594cf69bc4c55e27ac82032022-12-21T22:09:16ZengBMCOrphanet Journal of Rare Diseases1750-11722020-12-0115S214410.1186/s13023-020-01621-3French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides)Benjamin Terrier0Raphaël Darbon1Cécile-Audrey Durel2Eric Hachulla3Alexandre Karras4Hélène Maillard5Thomas Papo6Xavier Puechal7Grégory Pugnet8Thomas Quemeneur9Maxime Samson10Camille Taille11Loïc Guillevin12CollaboratorsInternal Medicine, CHU Cochin, AP-HPFrench Vasculitis AssociationInternal Medicine, CHU LyonInternal Medicine, CHU LilleNephrology, HEGP, AP-HPInternal Medicine, CHU LilleInternal Medicine, CHU Bichat, AP-HPInternal Medicine, CHU Cochin, AP-HPInternal Medicine, CHU ToulouseInternal Medicine, CH ValenciennesInternal Medicine, CHU DijonPulmonology, CHU Bichat, AP-HPInternal Medicine, CHU Cochin, AP-HPAbstract 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.https://doi.org/10.1186/s13023-020-01621-3 |
| spellingShingle | Benjamin Terrier Raphaël Darbon Cécile-Audrey Durel Eric Hachulla Alexandre Karras Hélène Maillard Thomas Papo Xavier Puechal Grégory Pugnet Thomas Quemeneur Maxime Samson Camille Taille Loïc Guillevin Collaborators French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides) Orphanet Journal of Rare Diseases |
| title | French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides) |
| title_full | French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides) |
| title_fullStr | French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides) |
| title_full_unstemmed | French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides) |
| title_short | French recommendations for the management of systemic necrotizing vasculitides (polyarteritis nodosa and ANCA-associated vasculitides) |
| title_sort | french recommendations for the management of systemic necrotizing vasculitides polyarteritis nodosa and anca associated vasculitides |
| url | https://doi.org/10.1186/s13023-020-01621-3 |
| work_keys_str_mv | AT benjaminterrier frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT raphaeldarbon frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT cecileaudreydurel frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT erichachulla frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT alexandrekarras frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT helenemaillard frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT thomaspapo frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT xavierpuechal frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT gregorypugnet frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT thomasquemeneur frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT maximesamson frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT camilletaille frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT loicguillevin frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides AT collaborators frenchrecommendationsforthemanagementofsystemicnecrotizingvasculitidespolyarteritisnodosaandancaassociatedvasculitides |