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Primary central nervous system vasculitis

MedGen UID:
419271
Concept ID:
C2930862
Disease or Syndrome
Synonyms: Primary angiitis of the central nervous system; Primary CNS vasculitis
SNOMED CT: Primary angiitis of central nervous system (699814001); Idiopathic angiitis of central nervous system (699814001); PACNS - primary angiitis of central nervous system (699814001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0015374
Orphanet: ORPHA140989

Definition

A rare, medium or small vessel vasculitis characterized by focal and/or diffuse neurologic symptoms due to a documented arteritic process in the central nervous system, in the absence of other identified underlying cause (infectious, systemic, other neurologic diseases, etc.). It presents with non-specific symptoms of headache, stroke or transient ischemic attacks with cognitive impairment, hemiplegia, weakness, and rarely, with cranial nerve involvement, seizures and ataxia. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary central nervous system vasculitis

Professional guidelines

PubMed

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
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
Salvarani C, Pipitone N, Hunder GG
Curr Opin Rheumatol 2016 Jan;28(1):21-8. doi: 10.1097/BOR.0000000000000229. PMID: 26599380

Recent clinical studies

Etiology

Salvarani C, Brown RD Jr, Christianson TJH, Huston J 3rd, Giannini C, Hunder GG
Semin Arthritis Rheum 2024 Oct;68:152506. Epub 2024 Jun 29 doi: 10.1016/j.semarthrit.2024.152506. PMID: 38970897
de Boysson H, Arquizan C, Touzé E, Zuber M, Boulouis G, Naggara O, Guillevin L, Aouba A, Pagnoux C
Stroke 2018 Aug;49(8):1946-1952. doi: 10.1161/STROKEAHA.118.021878. PMID: 29986936
Salvarani C, Brown RD Jr, Christianson TJH, Huston J 3rd, Ansell SM, Giannini C, Hunder GG
Neurology 2018 Mar 6;90(10):e847-e855. Epub 2018 Feb 2 doi: 10.1212/WNL.0000000000005062. PMID: 29429967
Giannini C, Salvarani C, Hunder G, Brown RD
Acta Neuropathol 2012 Jun;123(6):759-72. Epub 2012 Mar 16 doi: 10.1007/s00401-012-0973-9. PMID: 22421812
Zuccoli G, Pipitone N, Haldipur A, Brown RD Jr, Hunder G, Salvarani C
Clin Exp Rheumatol 2011 Jan-Feb;29(1 Suppl 64):S104-9. Epub 2011 May 11 PMID: 21586204

Diagnosis

Salvarani C, Hunder GG, Brown RD Jr
N Engl J Med 2024 Sep 19;391(11):1028-1037. doi: 10.1056/NEJMra2314942. PMID: 39292929
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
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
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
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

Salvarani C, Hunder GG, Brown RD Jr
N Engl J Med 2024 Sep 19;391(11):1028-1037. doi: 10.1056/NEJMra2314942. PMID: 39292929
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
Rice CM, Scolding NJ
Pract Neurol 2020 Apr;20(2):109-114. Epub 2019 Oct 24 doi: 10.1136/practneurol-2018-002002. PMID: 31649101
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
Salvarani C, Brown RD Jr, Christianson TJ, Huston J 3rd, Giannini C, Miller DV, Muratore F, Hunder GG
Semin Arthritis Rheum 2015 Aug;45(1):55-9. Epub 2015 Feb 20 doi: 10.1016/j.semarthrit.2015.02.008. PMID: 25800827

Prognosis

Rice CM, Scolding NJ
Pract Neurol 2020 Apr;20(2):109-114. Epub 2019 Oct 24 doi: 10.1136/practneurol-2018-002002. PMID: 31649101
Younger DS
Neurol Clin 2019 May;37(2):149-170. doi: 10.1016/j.ncl.2019.01.001. PMID: 30952403
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
Salvarani C, Pipitone N, Hunder GG
Curr Opin Rheumatol 2016 Jan;28(1):21-8. doi: 10.1097/BOR.0000000000000229. PMID: 26599380
Zuccoli G, Pipitone N, Haldipur A, Brown RD Jr, Hunder G, Salvarani C
Clin Exp Rheumatol 2011 Jan-Feb;29(1 Suppl 64):S104-9. Epub 2011 May 11 PMID: 21586204

Clinical prediction guides

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
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
Salvarani C, Pipitone N, Hunder GG
Curr Opin Rheumatol 2016 Jan;28(1):21-8. doi: 10.1097/BOR.0000000000000229. PMID: 26599380
Giannini C, Salvarani C, Hunder G, Brown RD
Acta Neuropathol 2012 Jun;123(6):759-72. Epub 2012 Mar 16 doi: 10.1007/s00401-012-0973-9. PMID: 22421812
Zuccoli G, Pipitone N, Haldipur A, Brown RD Jr, Hunder G, Salvarani C
Clin Exp Rheumatol 2011 Jan-Feb;29(1 Suppl 64):S104-9. Epub 2011 May 11 PMID: 21586204

Recent systematic reviews

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

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