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The most common causes of large-vessel vasculitis are giant cell arteritis and Takayasu arteritis, and those of medium-vessel arteritis are polyarteritis nodosa and Kawasaki disease. However, there is some overlap, and arteries of any size can potentially be involved in any of the 3 main categories of dominant vessel involvement. In addition to multisystem vasculitides, other forms of vasculitis have been defined, including single-organ vasculitis (eg, isolated aortitis). Prompt identification of vasculitides is important because they are associated with an increased risk of mortality. Left undiagnosed or mismanaged, these conditions may result in serious adverse outcomes that might otherwise have been avoided or minimized. The ethnic and regional differences in the incidence, prevalence, and clinical characteristics of patients with vasculitis should be recognized. Because the clinical presentation of vasculitis is highly variable, the cardiovascular clinician must have a high index of suspicion to establish a reliable and prompt diagnosis. This article reviews the pathophysiology, epidemiology, diagnostic strategies, and management of vasculitis.<\/jats:p>","DOI":"10.1161\/circulationaha.120.046657","type":"journal-article","created":{"date-parts":[[2021,1,19]],"date-time":"2021-01-19T14:01:05Z","timestamp":1611064865000},"page":"267-282","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":176,"title":["Medium- and Large-Vessel Vasculitis"],"prefix":"10.1161","volume":"143","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3628-9996","authenticated-orcid":true,"given":"David","family":"Saadoun","sequence":"first","affiliation":[{"name":"Sorbonne Universit\u00e9s, Assistance Publique H\u00f4pitaux de Paris (AP-HP), Piti\u00e9-Salp\u00eatri\u00e8re University Hospital, Department of Internal Medicine and Clinical Immunology, Centre national de R\u00e9f\u00e9rence des Maladies Auto-Immunes Syst\u00e9miques Rares, Centre national de R\u00e9f\u00e9rence des Maladies Auto-Inflammatoires et de l\u2019Amylose inflammatoire, Paris, France (D.S., M.V.)."},{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM), Unit\u00e9 mixte de recherche (UMR) S 959, and Recherche Hospitalo-Universitaire en sant\u00e9 (RHU) Interleukin-2 Therapy for autoimmune and inflammatory diseases, Paris, France (D.S., P.C.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathieu","family":"Vautier","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9s, Assistance Publique H\u00f4pitaux de Paris (AP-HP), Piti\u00e9-Salp\u00eatri\u00e8re University Hospital, Department of Internal Medicine and Clinical Immunology, Centre national de R\u00e9f\u00e9rence des Maladies Auto-Immunes Syst\u00e9miques Rares, Centre national de R\u00e9f\u00e9rence des Maladies Auto-Inflammatoires et de l\u2019Amylose inflammatoire, Paris, France (D.S., M.V.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrice","family":"Cacoub","sequence":"additional","affiliation":[{"name":"Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale (INSERM), Unit\u00e9 mixte de recherche (UMR) S 959, and Recherche Hospitalo-Universitaire en sant\u00e9 (RHU) Interleukin-2 Therapy for autoimmune and inflammatory diseases, Paris, France (D.S., P.C.)."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.37715"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.23176"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2017.04.001"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamaophthalmol.2019.2919"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.30155"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ket231"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1097\/WNO.0b013e318268aa9b"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kex428"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000180042.42156.d1"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ken465"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0000000000000265"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2011-200865"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.11353"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2013-204113"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1097\/MD.0b013e318206af16"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780330810"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keu329"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2017.11.029"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1136\/ard.46.4.282"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaa196"},{"key":"e_1_3_3_22_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.21699"},{"key":"e_1_3_3_23_2","first-page":"1","article-title":"An arteritis with special reference to polymyalgia arteritica.","volume":"237","author":"Ostberg G","year":"1973","unstructured":"Ostberg, G. 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