Systemic primary vasculitides are rare idiopathic diseases causing an inflammatory injury to the vessel walls.
G Serra, A -L Brun, D Toledano, P Cluzel, P A Grenier 2014, 'Review article: Thoracic involvement in systemic primary vasculitis: radiologicalpatterns and follow-up', Journal of the Belgian Society of Radiologyhttps://www.jbsr.be/articles/36. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)
It generally occurs in association with temporal arteritis and other vasculitides.
Fabio Muradás Girardi, Eduardo Lange Rech, Victória Teles França, Luiz Inácio Roman 2016, 'Tongue gangrene and oropharyngeal carcinoma', Clinical and Biomedical Researchhttp://seer.ufrgs.br/index.php/hcpa/article/view/66052. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)
Vasculitides are characterized by inflammation and necrosis of the blood vessel wall.
Peter Berlit 2010, 'Review: Diagnosis and treatment of cerebral vasculitis', Therapeutic Advances in Neurological Disordershttps://doi.org/10.1177/1756285609347123. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)
Different vasculitides with indistinguishable clinical presentations have very different prognosis and treatments.
Hebert Roberto Clivati Brandt, Marcelo Arnone, Neusa Yuriko Sakai Valente, Paulo RicardoCriado, Mirian Nacagami Sotto 2007, 'Vasculite cutânea de pequenos vasos: etiologia, patogênese, classificação e critériosdiagnósticos - Parte I Small vessel cutaneous vasculitis: etiology, pathogenesis,classification and diagnostic criteria - Part I', Anais Brasileiros de Dermatologiahttp://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962007000500002. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)
The epidemiology of systemic vasculitides differs widely among countries.
Takayuki Katsuyama, Ken-Ei Sada, Hirofumi Makino 2014, 'Current Concept and Epidemiology of Systemic Vasculitides', Allergology Internationalhttp://www.sciencedirect.com/science/article/pii/S1323893015300630. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)
The treatment recommendations for cerebral angitis are derived from protocols for systemic vasculitides.
Peter Berlit 2010, 'Review: Diagnosis and treatment of cerebral vasculitis', Therapeutic Advances in Neurological Disordershttps://doi.org/10.1177/1756285609347123. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)
Advances in understanding the biology of the vasculitides are improving therapies.
Raashid Ahmed Luqmani 2014, 'State of the art in the treatment of systemic vasculitides', Frontiers in Immunologyhttp://journal.frontiersin.org/Journal/10.3389/fimmu.2014.00471/full. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)
Cutaneous vasculitides in childhood are rare and often present with clinical features distinct from adults.
Palit Aparna, Inamadar Arun 2009, 'Childhood cutaneous vasculitis : A comprehensive appraisal', Indian Journal of Dermatologyhttp://www.e-ijd.org/article.asp?issn=0019-5154;year=2009;volume=54;issue=2;spage=110;epage=117;aulast=Palit. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)
Histologic examination of inner ear structures and other tissues revealed the presence of severe vascular lesions consistent with systemic vasculitides.
Kathleen A. Cashman, Eric R. Wilkinson, Xiankun Zeng, Anthony P. Cardile, Paul R.Facemire, Todd M. Bell, Jeremy J. Bearss, Carl I. Shaia, Connie S. Schmaljohn 2018, 'Immune-Mediated Systemic Vasculitis as the Proposed Cause of Sudden-Onset SensorineuralHearing Loss following Lassa Virus Exposure in Cynomolgus Macaques', mBiohttps://doi.org/10.1128/mBio.01896-18. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)
In large-vessel vasculitides, inflammatory infiltrates may cause thickening of the involved arterial vessel wall leading to progressive stenosis and occlusion.
Enrico eAmmirati, Enrico eAmmirati, Francesco eMoroni, Patrizia ePedrotti, IsabellaeScotti, Marco eMagnoni, Enrica P Bozzolo, Ornella E Rimoldi, Paolo eCamici 2014, 'Noninvasive imaging of vascular inflammation', Frontiers in Immunologyhttp://journal.frontiersin.org/Journal/10.3389/fimmu.2014.00399/full. Retrieved from DOAJ CC BY 4.0 (https://creativecommons.org/licenses/by-sa/4.0/legalcode)