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Central nervous system vasculitis

MedGen UID:
148408
•Concept ID:
C0751878
•
Disease or Syndrome
Synonyms: Angiitis, Central Nervous System; Central Nervous System Angiitis; Central Nervous System Vasculitis; CNS Vasculitis; Vasculitis, Central Nervous System; Vasculitis, CNS
 
Monarch Initiative: MONDO:0003346

Definition

Vasculitis affecting the blood vessels of the brain and/or spinal cord. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral nervous system vasculitis

Professional guidelines

PubMed

Selective immune suppression using interleukin-6 receptor inhibitors for management of immune-related adverse events.
Fa'ak F, Buni M, Falohun A, Lu H, Song J, Johnson DH, Zobniw CM, Trinh VA, Awiwi MO, Tahon NH, Elsayes KM, Ludford K, Montazari EJ, Chernis J, Dimitrova M, Sandigursky S, Sparks JA, Abu-Shawer O, Rahma O, Thanarajasingam U, Zeman AM, Talukder R, Singh N, Chung SH, Grivas P, Daher M, Abudayyeh A, Osman I, Weber J, Tayar JH, Suarez-Almazor ME, Abdel-Wahab N, Diab A
J Immunother Cancer 2023 Jun;11(6) doi: 10.1136/jitc-2023-006814. PMID: 37328287Free PMC Article
Primary central nervous system vasculitis - An update on diagnosis, differential diagnosis and treatment.
Kraemer M, Berlit P
J Neurol Sci 2021 May 15;424:117422. Epub 2021 Mar 27 doi: 10.1016/j.jns.2021.117422. PMID: 33832773
CNS Vasculitis: an Approach to Differential Diagnosis and Management.
Byram K, Hajj-Ali RA, Calabrese L
Curr Rheumatol Rep 2018 May 30;20(7):37. doi: 10.1007/s11926-018-0747-z. PMID: 29846828

Recent clinical studies

Etiology

Fatal iatrogenic cerebral β-amyloid-related arteritis in a woman treated with lecanemab for Alzheimer's disease.
Solopova E, Romero-Fernandez W, Harmsen H, Ventura-Antunes L, Wang E, Shostak A, Maldonado J, Donahue MJ, Schultz D, Coyne TM, Charidimou A, Schrag M
Nat Commun 2023 Dec 12;14(1):8220. doi: 10.1038/s41467-023-43933-5. PMID: 38086820Free PMC Article
Selective immune suppression using interleukin-6 receptor inhibitors for management of immune-related adverse events.
Fa'ak F, Buni M, Falohun A, Lu H, Song J, Johnson DH, Zobniw CM, Trinh VA, Awiwi MO, Tahon NH, Elsayes KM, Ludford K, Montazari EJ, Chernis J, Dimitrova M, Sandigursky S, Sparks JA, Abu-Shawer O, Rahma O, Thanarajasingam U, Zeman AM, Talukder R, Singh N, Chung SH, Grivas P, Daher M, Abudayyeh A, Osman I, Weber J, Tayar JH, Suarez-Almazor ME, Abdel-Wahab N, Diab A
J Immunother Cancer 2023 Jun;11(6) doi: 10.1136/jitc-2023-006814. PMID: 37328287Free PMC Article
Vasculitis in the Central Nervous System.
Bougea A, Spantideas N
Adv Exp Med Biol 2017;1003:173-185. doi: 10.1007/978-3-319-57613-8_9. PMID: 28667559
Pediatric vasculitis.
Weiss PF
Pediatr Clin North Am 2012 Apr;59(2):407-23. Epub 2012 Apr 6 doi: 10.1016/j.pcl.2012.03.013. PMID: 22560577Free PMC Article
CNS vasculitis.
Kelley RE
Front Biosci 2004 Jan 1;9:946-55. doi: 10.2741/1259. PMID: 14766421

Diagnosis

Diagnostic and therapeutic approach to adult central nervous system vasculitis.
Nehme A, Boulanger M, Aouba A, Pagnoux C, Zuber M, Touzé E, de Boysson H
Rev Neurol (Paris) 2022 Dec;178(10):1041-1054. Epub 2022 Sep 22 doi: 10.1016/j.neurol.2022.05.003. PMID: 36156251
Primary central nervous system vasculitis - An update on diagnosis, differential diagnosis and treatment.
Kraemer M, Berlit P
J Neurol Sci 2021 May 15;424:117422. Epub 2021 Mar 27 doi: 10.1016/j.jns.2021.117422. PMID: 33832773
Autoimmune Encephalitides.
Younger DS
Neurol Clin 2019 May;37(2):359-381. Epub 2019 Mar 16 doi: 10.1016/j.ncl.2019.01.015. PMID: 30952414
CNS Vasculitis: an Approach to Differential Diagnosis and Management.
Byram K, Hajj-Ali RA, Calabrese L
Curr Rheumatol Rep 2018 May 30;20(7):37. doi: 10.1007/s11926-018-0747-z. PMID: 29846828
Adult primary central nervous system vasculitis.
Salvarani C, Brown RD Jr, Hunder GG
Lancet 2012 Aug 25;380(9843):767-77. Epub 2012 May 9 doi: 10.1016/S0140-6736(12)60069-5. PMID: 22575778

Therapy

Primary central nervous system vasculitis - An update on diagnosis, differential diagnosis and treatment.
Kraemer M, Berlit P
J Neurol Sci 2021 May 15;424:117422. Epub 2021 Mar 27 doi: 10.1016/j.jns.2021.117422. PMID: 33832773
CNS Vasculitis: an Approach to Differential Diagnosis and Management.
Byram K, Hajj-Ali RA, Calabrese L
Curr Rheumatol Rep 2018 May 30;20(7):37. doi: 10.1007/s11926-018-0747-z. PMID: 29846828
Clinical management of infectious cerebral vasculitides.
Carod Artal FJ
Expert Rev Neurother 2016;16(2):205-21. Epub 2016 Jan 19 doi: 10.1586/14737175.2015.1134321. PMID: 26689107
Pediatric vasculitis.
Weiss PF
Pediatr Clin North Am 2012 Apr;59(2):407-23. Epub 2012 Apr 6 doi: 10.1016/j.pcl.2012.03.013. PMID: 22560577Free PMC Article
Autoimmune encephalopathy.
Flanagan EP, Caselli RJ
Semin Neurol 2011 Apr;31(2):144-57. Epub 2011 May 17 doi: 10.1055/s-0031-1277985. PMID: 21590620

Prognosis

Central nervous system vasculitis in adults: An update.
Dutra LA, de Souza AW, Grinberg-Dias G, Barsottini OG, Appenzeller S
Autoimmun Rev 2017 Feb;16(2):123-131. Epub 2017 Jan 11 doi: 10.1016/j.autrev.2016.12.001. PMID: 28087351
Pediatric vasculitis.
Barut K, Sahin S, Kasapcopur O
Curr Opin Rheumatol 2016 Jan;28(1):29-38. doi: 10.1097/BOR.0000000000000236. PMID: 26555448
Biomarkers in vasculitis.
Monach PA
Curr Opin Rheumatol 2014 Jan;26(1):24-30. doi: 10.1097/BOR.0000000000000009. PMID: 24257367Free PMC Article
Autoimmune encephalopathy.
Flanagan EP, Caselli RJ
Semin Neurol 2011 Apr;31(2):144-57. Epub 2011 May 17 doi: 10.1055/s-0031-1277985. PMID: 21590620
CNS vasculitis.
Kelley RE
Front Biosci 2004 Jan 1;9:946-55. doi: 10.2741/1259. PMID: 14766421

Clinical prediction guides

Primary central nervous system vasculitis - An update on diagnosis, differential diagnosis and treatment.
Kraemer M, Berlit P
J Neurol Sci 2021 May 15;424:117422. Epub 2021 Mar 27 doi: 10.1016/j.jns.2021.117422. PMID: 33832773
RCVS(2) score and diagnostic approach for reversible cerebral vasoconstriction syndrome.
Rocha EA, Topcuoglu MA, Silva GS, Singhal AB
Neurology 2019 Feb 12;92(7):e639-e647. Epub 2019 Jan 11 doi: 10.1212/WNL.0000000000006917. PMID: 30635475
CNS Vasculitis: an Approach to Differential Diagnosis and Management.
Byram K, Hajj-Ali RA, Calabrese L
Curr Rheumatol Rep 2018 May 30;20(7):37. doi: 10.1007/s11926-018-0747-z. PMID: 29846828
Management of primary and secondary central nervous system vasculitis.
Salvarani C, Pipitone N, Hunder GG
Curr Opin Rheumatol 2016 Jan;28(1):21-8. doi: 10.1097/BOR.0000000000000229. PMID: 26599380
Biomarkers in vasculitis.
Monach PA
Curr Opin Rheumatol 2014 Jan;26(1):24-30. doi: 10.1097/BOR.0000000000000009. PMID: 24257367Free PMC Article

Recent systematic reviews

COVID-19 associated pediatric vasculitis: A systematic review and detailed analysis of the pathogenesis.
Batu ED, Sener S, Ozen S
Semin Arthritis Rheum 2022 Aug;55:152047. Epub 2022 Jun 9 doi: 10.1016/j.semarthrit.2022.152047. PMID: 35709649Free PMC Article
Vessel wall MR imaging of central nervous system vasculitis: a systematic review.
Arnett N, Pavlou A, Burke MP, Cucchiara BL, Rhee RL, Song JW
Neuroradiology 2022 Jan;64(1):43-58. Epub 2021 May 3 doi: 10.1007/s00234-021-02724-9. PMID: 33938989
Primary Angiitis of the CNS: A Systematic Review and Meta-analysis.
Beuker C, Strunk D, Rawal R, Schmidt-Pogoda A, Werring N, Milles L, Ruck T, Wiendl H, Meuth S, Minnerup H, Minnerup J
Neurol Neuroimmunol Neuroinflamm 2021 Nov;8(6) Epub 2021 Oct 18 doi: 10.1212/NXI.0000000000001093. PMID: 34663675Free PMC Article
Primary CNS vasculitis: A systematic review on clinical characteristics associated with abnormal biopsy and angiography.
Krawczyk M, Barra LJ, Sposato LA, Mandzia JL
Autoimmun Rev 2021 Jan;20(1):102714. Epub 2020 Nov 13 doi: 10.1016/j.autrev.2020.102714. PMID: 33197577
Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis--a review.
Pistracher K, Gellner V, Riegler S, Schökler B, Scarpatetti M, Kurschel S
Childs Nerv Syst 2012 Aug;28(8):1141-8. Epub 2012 Jun 9 doi: 10.1007/s00381-012-1827-4. PMID: 22684518

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