Lesion of central nervous system in vasculitis

Yu.N. Sorokin


The article presents the classification of vasculitis with central nervous system (CNS) lesions, their clinical manifestations, diagnostic criteria of primary CNS vasculitis and treatment methods. The diagnosis of primary vasculitis of the CNS requires, above all, the exclusion of the secondary nature of vascular lesions of the brain. In some cases, there may be certain situations which require the revision of a previously made diagnosis. A clinical case with a possible revision of the previously diagnosed multiple sclerosis in favor of the secondary cerebral vasculitis on the background of autoimmune thyroiditis is also discussed in the article.


central nervous system vasculitis; angiitis; stroke; headache; cognitive dysfunction; magnetic resonance imaging, angiography; steroids; multiple sclerosis; ICD-11


Jennette J.C., Falk R.J., Bacon P.A. et al. 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013. 65. 1-11.

ICD-11 for Mortality and Morbidity Statistics (2018). Accessed November 21, 2018.

Hoffman G.S., Calabrese L.H. Vasculitis: determinants of di-sease patterns. Nat. Rev. Rheumatol. 2014. 10. 454-462.

Salvarani C., Brown R.D., Christianson T.J.H. et al. An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: description of 163 patients. Medicine. 2015. 94. e738.

Тотолян Н.А., Готовчиков А.А., Кодзаева А.Ю. и др. Диагностика изолированного ангиита центральной нервной системы. Журнал неврологии и психиатрии им. С.С. Корсакова. 2013. 2(2). 32-42.

Hajj-Ali R.A., Calabrese L.H. Diagnosis and classification of central nervous system vasculitis. J. Autoimmun. 2014. 48–49. 149-152.

Twilt M., Benseler S.M. Central nervous system vasculitis in adults and children. Handbook of Clinical Neurology. Elsevier. 2016. 133. 283-300.

Wan Ch., Su H. A Closer Look at Angiitis of the central nervous system. Neurosciences (Riyadh). 2017. 22(4). 247-254.

Giannini C., Salvarani C., Hunder G., Brown R.D. Primary central nervous system vasculitis: pathology and mechanisms. Acta Neuropathol. 2012. 123. 759-772.

Ruland T., Wolbert J., Gottschalk M.G. et al. Cerebrospinal Fluid Concentrations of Neuronal Proteins Are Reduced in Primary Angiitis of the Central Nervous System. Front Neurol. 2018. 9. 407-416.

Hajj-Ali R.A., Singhal A.B., Benseler S. et al. Primary angiitis of the CNS. The Lancet Neurology. 2011. 10(6). 561-572.

De Boysson H., Boulouis G., Aouba A. et al. Adult primary angiitis of the central nervous system: isolated small vessel vasculitis represents distinct disease pattern. Rheumatology (Oxford). 2017. 56. 439-444.

Beuker С., Schmidt A., Strunk D. et al. Primary angiitis of the central nervous system: diagnosis and treatment. Ther. Adv. Neurol. Disord. 2018. 11. 1756286418785071.

Kumar P.P., Rajesh A., Kandadai R.M. et al. Primary CNS vasculitis masquerading as glioblastoma: A case report and review. Asian J. Neurosurg. 2017. 12. 69-71.

Razek A.A.Kh.A., Alvarez H., Bagg S. et al. Imaging Spectrum of CNS Vasculitis. Radio Graphics. 2014. 34 873-894.

Salvarani C., Brown R.D., Christianson T.J.H. et al. Primary central nervous system vasculitis mimicking brain tumor: Comprehensive analysis of 13 cases from a single institutional cohort of 191 cases. J. of Autoimmunity. 2019. 97. 22-28. Available online 24 October 2018.

Nocton J.J. Usual and Unusual Manifestations of systemic and central nervous system vasculitis. Pediatr Clin. N. Am. 2017. 64. 185-204.

De Boysson H., Boulouis G., Parienti J.-J. et al. Concordance of Time-of-Flight MRA and Digital Subtraction Angiography in Adult Primary Central Nervous System Vasculitis. Am. J. Neuroradiol. 2017. 38(10). 1917-1922.

Hammad T.A., Hajj-Ali R.A. Primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome. Curr. Atheroscler. Rep. 2013. 15(8). 346-354.

Niu L., Wang L., Yin X. et al. Role of magnetic resonance imaging in the diagnosis of primary central nervous system angiitis. Experimental and Therapeutic Medicine. 2017. 14. 555-560.

Chimelli L. The role of biopsies and autopsies in the diagnosis of cognitive impairment, with emphasis on small vessel diseases: A critical appraisal enriched by personal experience. Dement. Neuropsychol. 2017. 11(4). 356-363.

Lucke M., Hajj-Ali R.A. Advances in primary angiitis of the central nervous system. Curr. Cardiol. Rep. 2014. 16(10). 533-539.

Dutra L.A., de Souza A.W.S., Grinberg-Dias G. et al. Central nervous system vasculitis in adults: An update. Autoimmunity Reviews. 2017. 16(2). 123-131.

Vera-Lastra О., Sepúlveda-Delgado J., Cruz-Domínguez M.Р. et al. Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis. Clin. Rheumatol. 2015. 34(4). 729-738.

Becker J., Horn P.A., Keyvani K. et al. Primary central nervous system vasculitis and its mimicking diseases — clinical features, outcome, comorbidities and diagnostic results — A case control study. Clinical Neurology and Neurosurgery. 2017. 156. 48-54.

Киселева Е.В., Спирин Н.Н. Сочетание рассеянного склероза и аутоиммунной патологии щитовидной железы: особенности течения демиелинизирующего процесса. Практическая медицина. 2017. 1(102). 92-95.

Шмонина И.А., Шмонин А.А., Красильникова Е.И., Тотолян Н.А. Аутоиммунные заболевания щитовидной железы у пациентов с рассеянным склерозом: возможные взаимосвязи. Ученые записки СПбГМУ им. И.П. Павлова. 2012. 19(1). 11-16.

Ntatsaki E., Carruthers D., Chakravarty K. et al. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014. 53(12). 2306-2309.

Salvarani C., Brown R.D., Christianson T.J.H. et al. Adult primary central nervous system vasculitis treatment and course: ana-lysis of one hundred sixty-three patients. Arthritis & Rheumatology. 2015. 67(6). 1637-1645.

Yates M., Watts R.A., Bajema I.M. et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann. Rheum. Dis. 2016. 75(9). 1583-1594.

De Boysson H., Parienti J.J., Arquizan C. et al. Maintenance therapy is associated with better long-term outcomes in adult patients with primary angiitis of the central nervous system. Rheumatology (Oxford). 2017. 56(10). 1684-1693.

Salvarani C., Brown R.D., Giannini C., Hunder G.G. Adult Primary Central Nervous System Vasculitis. Systemic Vasculitides: Current Status and Perspectives. 2016. 245-257.

Бекетова Т.В. Международные рекомендации по лечению АНЦА-ассоциированных системных васкулитов. Современная ревматология. 2019. 13(1). 19-25.

Deng J., Fang F., Wang X.H. et al. Small vessel-childhood primary angiitis of the central nervous system: a case report and literature review. Zhonghua Er Ke Za Zhi. 2018. 56(2). 142-147.

Twilt M., Benseler S.M. Treatment of CNS Vasculitis in Children. Curr. Treat. Options in Rheum. 2015. 1. 365-380.

Hajj-Ali R.A., Saygin D., Ray E. et al. Long-term outcomes of patients with primary angiitis of the central nervous system. Clin. Exp. Rheumatol. 2019. 37(Suppl. 117(2)). 45-51.


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