U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Vasculitis of systemic vasculature

MedGen UID:
82688
Concept ID:
C0264939
Disease or Syndrome
Synonyms: Systemic Vasculitides; Systemic Vasculitis; Vasculitides, Systemic; Vasculitis, Systemic
SNOMED CT: Vasculitis of systemic vasculature (1268294009); Inflammation of systemic vasculature (1268294009); Systemic vasculitis (1268294009)

Definition

A heterogeneous group of diseases characterized by inflammation and necrosis of the blood vessel walls. [from MeSH]

Professional guidelines

PubMed

Kronbichler A, Bajema IM, Bruchfeld A, Mastroianni Kirsztajn G, Stone JH
Lancet 2024 Feb 17;403(10427):683-698. doi: 10.1016/S0140-6736(23)01736-1. PMID: 38368016
Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Ozen S, Marks SD, Brogan P, Groot N, de Graeff N, Avcin T, Bader-Meunier B, Dolezalova P, Feldman BM, Kone-Paut I, Lahdenne P, McCann L, Pilkington C, Ravelli A, van Royen A, Uziel Y, Vastert B, Wulffraat N, Kamphuis S, Beresford MW
Rheumatology (Oxford) 2019 Sep 1;58(9):1607-1616. doi: 10.1093/rheumatology/kez041. PMID: 30879080

Recent clinical studies

Etiology

Cottu A, Groh M, Desaintjean C, Marchand-Adam S, Guillevin L, Puechal X, Beaumesnil S, Lazaro E, Samson M, Taille C, Durel CA, Diot E, Nicolas S, Guilleminault L, Ebbo M, Cathebras P, Dupin C, Yildiz H, Belfeki N, Pugnet G, Chauvin P, Jouneau S, Lifermann F, Martellosio JP, Cottin V, Terrier B; French Vasculitis Study Group
Ann Rheum Dis 2023 Dec;82(12):1580-1586. Epub 2023 Aug 7 doi: 10.1136/ard-2023-224624. PMID: 37550002
Kermani TA, Warrington KJ, Dua AB
Rheum Dis Clin North Am 2022 Aug;48(3):705-724. Epub 2022 Jul 5 doi: 10.1016/j.rdc.2022.03.006. PMID: 35953232
Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22 PMID: 32324122
Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Toumelin PL; French Vasculitis Study Group (FVSG)
Medicine (Baltimore) 2011 Jan;90(1):19-27. doi: 10.1097/MD.0b013e318205a4c6. PMID: 21200183
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, Buoncompagni A, Lazar C, Bilge I, Uziel Y, Rigante D, Cantarini L, Hilario MO, Silva CA, Alegria M, Norambuena X, Belot A, Berkun Y, Estrella AI, Olivieri AN, Alpigiani MG, Rumba I, Sztajnbok F, Tambic-Bukovac L, Breda L, Al-Mayouf S, Mihaylova D, Chasnyk V, Sengler C, Klein-Gitelman M, Djeddi D, Nuno L, Pruunsild C, Brunner J, Kondi A, Pagava K, Pederzoli S, Martini A, Ruperto N; Paediatric Rheumatology International Trials Organisation (PRINTO)
Ann Rheum Dis 2010 May;69(5):798-806. doi: 10.1136/ard.2009.116657. PMID: 20413568

Diagnosis

Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Moretti M, Treppo E, Monti S, La Rocca G, Del Frate G, Delvino P, Italiano N, Di Cianni F, D'Alessandro F, Talarico R, Ferro F, Quartuccio L, Baldini C
Clin Exp Rheumatol 2023 Apr;41(4):765-773. Epub 2023 Apr 18 doi: 10.55563/clinexprheumatol/zf4daj. PMID: 37073639
Romero Gómez C, Hernández Negrín H, Ayala Gutiérrez MDM
Med Clin (Barc) 2023 Apr 6;160(7):310-317. Epub 2023 Feb 9 doi: 10.1016/j.medcli.2023.01.003. PMID: 36774291
Hočevar A, Tomšič M, Perdan Pirkmajer K
Curr Rheumatol Rep 2021 Feb 10;23(3):14. doi: 10.1007/s11926-021-00983-2. PMID: 33569653
Frumholtz L, Laurent-Roussel S, Lipsker D, Terrier B
Clin Rev Allergy Immunol 2021 Oct;61(2):181-193. doi: 10.1007/s12016-020-08788-4. PMID: 32378145

Therapy

Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Torp CK, Brüner M, Keller KK, Brouwer E, Hauge EM, McGonagle D, Kragstrup TW
Autoimmun Rev 2021 Jun;20(6):102829. Epub 2021 Apr 16 doi: 10.1016/j.autrev.2021.102829. PMID: 33872767
Hočevar A, Tomšič M, Perdan Pirkmajer K
Curr Rheumatol Rep 2021 Feb 10;23(3):14. doi: 10.1007/s11926-021-00983-2. PMID: 33569653
Puéchal X
Joint Bone Spine 2020 Dec;87(6):572-578. Epub 2020 Jun 17 doi: 10.1016/j.jbspin.2020.06.005. PMID: 32562697
Hernández-Rodríguez J, Alba MA, Prieto-González S, Cid MC
J Autoimmun 2014 Feb-Mar;48-49:84-9. Epub 2014 Jan 28 doi: 10.1016/j.jaut.2014.01.029. PMID: 24485157

Prognosis

Springer JM, Byram K
Postgrad Med 2023 Jan;135(sup1):61-68. Epub 2022 Jun 22 doi: 10.1080/00325481.2022.2088940. PMID: 35709399
Bilginer Y, Ozen S
Curr Opin Pediatr 2022 Apr 1;34(2):229-233. doi: 10.1097/MOP.0000000000001106. PMID: 35081554
Hočevar A, Tomšič M, Perdan Pirkmajer K
Curr Rheumatol Rep 2021 Feb 10;23(3):14. doi: 10.1007/s11926-021-00983-2. PMID: 33569653
Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22 PMID: 32324122
Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M
Int J Immunopathol Pharmacol 2016 Jun;29(2):151-9. Epub 2015 Dec 18 doi: 10.1177/0394632015617063. PMID: 26684637Free PMC Article

Clinical prediction guides

Cottu A, Groh M, Desaintjean C, Marchand-Adam S, Guillevin L, Puechal X, Beaumesnil S, Lazaro E, Samson M, Taille C, Durel CA, Diot E, Nicolas S, Guilleminault L, Ebbo M, Cathebras P, Dupin C, Yildiz H, Belfeki N, Pugnet G, Chauvin P, Jouneau S, Lifermann F, Martellosio JP, Cottin V, Terrier B; French Vasculitis Study Group
Ann Rheum Dis 2023 Dec;82(12):1580-1586. Epub 2023 Aug 7 doi: 10.1136/ard-2023-224624. PMID: 37550002
Koike H, Nishi R, Yagi S, Furukawa S, Fukami Y, Iijima M, Katsuno M
Adv Ther 2023 Jan;40(1):25-40. Epub 2022 Sep 24 doi: 10.1007/s12325-022-02307-x. PMID: 36152266
Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F
Clin Exp Rheumatol 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22 PMID: 32324122
Comarmond C, Cacoub P
Autoimmun Rev 2014 Nov;13(11):1121-5. Epub 2014 Aug 20 doi: 10.1016/j.autrev.2014.08.017. PMID: 25149391
Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, Flossmann O, Hall C, Hollywood J, Jayne D, Jones R, Lanyon P, Muir A, Scott D, Young L, Luqmani RA
Ann Rheum Dis 2009 Dec;68(12):1827-32. Epub 2008 Dec 3 doi: 10.1136/ard.2008.101279. PMID: 19054820

Recent systematic reviews

Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D
Ann Rheum Dis 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764. PMID: 36927642
Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910Free PMC Article
Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Rheumatol 2021 Aug;73(8):1366-1383. Epub 2021 Jul 8 doi: 10.1002/art.41773. PMID: 34235894
Chung SA, Gorelik M, Langford CA, Maz M, Abril A, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot M, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA
Arthritis Care Res (Hoboken) 2021 Aug;73(8):1061-1070. Epub 2021 Jul 8 doi: 10.1002/acr.24633. PMID: 34235889
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...