Vasculitis in the Central Nervous System

Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 1003)


Central nervous system (CNS) vasculitides are a heterogeneous group of disorders characterized by an inflammatory cell infiltration and necrosis of blood vessel walls in the brain, spinal cord, and the meninges. The CNS complications are likely to be fatal without judicious use of immunosuppression; thus, early diagnosis may prevent from damage and disability. This chapter updates our knowledge on CNS vasculitis-related immunological mechanisms, neurological complications, diagnosis, and management.


Central nervous system (CNS) vasculitides Immunosuppression Immunological mechanisms Diagnosis Management 


  1. 1.
    Riggs JE. Neurological consequences of systemic disease. In: Bradley W, Daroff R, Fenichel G, Marsden D, editors. Neurology in clinical practice. Boston: Butterworth-Heinemann; 1991. p. 841–60.Google Scholar
  2. 2.
    Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11.CrossRefPubMedGoogle Scholar
  3. 3.
    Salvarani C, Cantini F, Boiardi L, Hunder GG. Polymyalgia rheumatica and giant-cell arteritis. N Engl J Med. 2002;347:261–71.CrossRefPubMedGoogle Scholar
  4. 4.
    Weyand C, Joyce Liao Y, Goronzy JJ. The immunopathology of giant cell arteritis: diagnostic and therapeutic implications. J Neuroophthalmol. 2012;32(3):259–65.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Nesher G. Neurologic manifestations of giant cell arteritis. Clin Exp Rheumatol. 2000;4(Suppl 20):S24–6.Google Scholar
  6. 6.
    Hoffman GS, Cid MC, Rendt-Zagar KE, Merkel PA, Weyand CM, Stone JH, et al. Infliximab-GCA study group. Infliximab for maintenance of glucocorticosteroid-induced remission of giant cell arteritis: a randomized trial. Ann Intern Med. 2007;146(9):621–30.CrossRefPubMedGoogle Scholar
  7. 7.
    Pfadenhauer K, Roesler A, Golling A. The involvement of the peripheral nervous system in biopsy proven active giant cell arteritis. J Neurol. 2007;254(6):751–5.CrossRefPubMedGoogle Scholar
  8. 8.
    Vishwanath S, Relan M, Shen L, Ambrus JL Jr. Update on the use of biologics in vasculitides. Curr Pharm Biotechnol. 2014;15(6):558–62.CrossRefPubMedGoogle Scholar
  9. 9.
    Seko Y, Takahashi N, Tada Y, Yaqida H, Okumura K, Naqai R. Restricted usage of T-cell receptor Vγ-Vδ genes and expression of co-stimulatory molecules in Takayasu's arteritis. Int J Cardiol. 2000;75:S77–83.CrossRefPubMedGoogle Scholar
  10. 10.
    Forbess L, Bannykh S. Polyarteritis nodosa. Rheum Dis Clin N Am. 2015;41(1):33–46.CrossRefGoogle Scholar
  11. 11.
    Chalk C, Dyck J, Conn D. Vasculitic neuropathy. In: Thomas PK, Dyck PJ, editors. Peripheral neuropathy. Philadelphia: Saunders; 1993. p. 1424–143.Google Scholar
  12. 12.
    Midroni G, Bilbao J. Vasculitic neuropathy. In: Midroni G, Bilbao J, editors. Biopsy diagnosis of peripheral neuropathy, vol. 1995. Boston: Butterworth-Heinemann; 1995. p. 241–62.CrossRefGoogle Scholar
  13. 13.
    Younger DS. Vasculitis of the nervous system. Curr Opin Neurol. 2004;17(3):317–36.CrossRefPubMedGoogle Scholar
  14. 14.
    Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33(8):1094–100.CrossRefPubMedGoogle Scholar
  15. 15.
    Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, et al. Churg- Strauss syndrome. Autoimmun Rev. 2015;14(4):341–8.CrossRefPubMedGoogle Scholar
  16. 16.
    Wolf J, Bergner R, Mutallib S, Buggle F, Grau AJ. Neurologic complications of Churg-Strauss syndrome--a prospective monocentric study. Eur J Neurol. 2010;17(4):582–8.CrossRefPubMedGoogle Scholar
  17. 17.
    McKinney EF, Willcocks LC, Broecker V, Smith KGC. The immunopatology of ANCA- associated vasculitis. Semin Immunopathol. 2014;36(4):461–78.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33(8):1101–7.CrossRefPubMedGoogle Scholar
  19. 19.
    Nishino H, Rubino FA, Parisi JE. The spectrum of neurologic involvement in Wegener’s granulomatosis. Neurology. 1993;43(7):1334.CrossRefPubMedGoogle Scholar
  20. 20.
    de Menthon M, Cohen P, Pagnoux C, Buchler M, Sibilia J, Détrée F, et al. Infliximab or rituximab for refractory Wegener’s granulomatosis: long-term follow-up. A prospective randomised multicentre study on 17 patients. Clin Exp Rheumatol. 2011;29(1Suppl 64):S63–71.PubMedGoogle Scholar
  21. 21.
    Morgan MD, Drayson MT, Savage CO, Harper L. Addition of infliximab to standard therapy for ANCA-associated vasculitis. Nephron Clin Pract. 2011;117(2):c89–97.CrossRefPubMedGoogle Scholar
  22. 22.
    International Team for the Revision of the International Criteria for Behcet’s Disease. Revision of the International criteria for Behcet’s disease (ICBD). Clin Exp Rheumatol. 2006;42:S14–5.Google Scholar
  23. 23.
    Dinc A, Erdem H, Simsek I, Pay S. Immunopathogenesis of Behcet’s disease with special emphasize on the possible role of antigen presenting cells. Rheumatol Int. 2007;27:417–24.CrossRefPubMedGoogle Scholar
  24. 24.
    Kidd D. Neurological complications of Behçet’s syndrome. Curr Neurol Neurosci Rep. 2012;12(6):675–9.CrossRefPubMedGoogle Scholar
  25. 25.
    Kikuchi H, Aramaki K, Hirohata S. Low dose MTX for progressive neuro-Behçet’s disease. A follow-up study for 4 years. Adv Exp Med Biol. 2003;528:575–8.CrossRefPubMedGoogle Scholar
  26. 26.
    Saip S, Akman-Demir G, Siva A. Neuro-Behçet syndrome. Handb Clin Neurol. 2014;121:1703–23.CrossRefPubMedGoogle Scholar
  27. 27.
    Kararizou E, Davaki P, Karandreas N, Davou R, Vassilopoulos D. Nonsystemic vasculitic neuropathy: a clinicopathological study of 22 cases. J Rheumatol. 2005;32(5):853–8.PubMedGoogle Scholar
  28. 28.
    Hammad TA, Hajj-Ali RA. Primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome. Curr Atheroscler Rep. 2013;15(8):346.CrossRefPubMedGoogle Scholar
  29. 29.
    Alba MA, Espígol-Frigolé G, Prieto-González S, Tavera-Bahillo I, Martínez AG, Butjosa M, et al. Central nervous system vasculitis: still more questions than answers. Curr Neuropharmacol. 2011;9:437–48.CrossRefPubMedPubMedCentralGoogle Scholar
  30. 30.
    Salvarani C, Brown RD Jr, Christianson TJ, et al. Adult primary central nervous system vasculitis treatment and course: analysis of one hundred sixty-three patients. Arthritis Rheumatol. 2015;67:1637–45.CrossRefPubMedGoogle Scholar
  31. 31.
    De Boysson H, Zuber M, Naggara O, et al. French Vasculitis Study Group and the French NeuroVascular Society. Primary angiitis of the central nervous system: description of the first fifty-two adults enrolled in the French cohort of patients with primary vasculitis of the central nervous system. Arthritis Rheumatol. 2014;66:1315–26.CrossRefPubMedGoogle Scholar
  32. 32.
    Choy E. Understanding the dynamics: pathways involved in the pathogenesis of rheumatoid arthritis. Rheumatology. 2012;51(suppl 5):v3–v11.CrossRefPubMedGoogle Scholar
  33. 33.
    Rosenbaum R. Neuromuscular complications of connective tissue diseases. Muscle Nerve. 2001;24(2):154–69.CrossRefPubMedGoogle Scholar
  34. 34.
    Caballol Pons N, Montalà N, Valverde J, Brell M, Ferrer I, Martínez-Yélamos S. Isolated cerebral vasculitis associated with rheumatoid arthritis. Joint Bone Spine. 2010;77(4):361–3.CrossRefPubMedGoogle Scholar
  35. 35.
    da Côrte FC, Neves N. Cervical spine instability in rheumatoid arthritis. Eur J Orthop Surg Traumatol. 2014;24(Suppl 1):S83–91.PubMedGoogle Scholar
  36. 36.
    Matsumoto T, Nakamura I, Miura A, Momoyama G, Ito K. New-onset multiple sclerosis associated with adalimumab treatment in rheumatoid arthritis: a case report and literature review. Clin Rheumatol. 2013;32(2):271–5.CrossRefPubMedGoogle Scholar
  37. 37.
    Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40(9):1725.CrossRefPubMedGoogle Scholar
  38. 38.
    Greenberg BM. The neurologic manifestations of systemic lupus erythematosus. Neurologist. 2009;15(3):115–21.CrossRefPubMedGoogle Scholar
  39. 39.
    Streifler JY, Molad Y. Connective tissue disorders: systemic lupus erythematosus, Sjögren’s syndrome, and scleroderma. Handb Clin Neurol. 2014;119:463–73.CrossRefPubMedGoogle Scholar
  40. 40.
    Wigren M, Nilsson J, Kaplan MJ. Pathogenic immunity in systemic lupus erythematosus and atherosclerosis: common mechanisms and possible targets for intervention. J Intern Med. 2015;278:494–506.CrossRefPubMedPubMedCentralGoogle Scholar
  41. 41.
    Jeltsch-David H, Muller S. Neuropsychiatric systemic lupus erythematosus: pathogenesis and biomarkers. Nat Rev Neurol. 2014;10(10):579–96.CrossRefPubMedGoogle Scholar
  42. 42.
    Zuber JP, Spertini F. Immunological basis of systemic sclerosis. Rheumatology. 2006;45(suppl 3):iii23–5.PubMedGoogle Scholar
  43. 43.
    Allanore Y, Kahan A. Primary myocardial involvement in systemic sclerosis. Rheumatology. 2006;45:iv14–7.PubMedGoogle Scholar
  44. 44.
    Amaral TN, Peres FA, Lapa AT, Marques-Neto JF, Appenzeller S. Neurologic involvement in scleroderma: a systematic review. Semin Arthritis Rheum. 2013;43(3):335–47.CrossRefPubMedGoogle Scholar
  45. 45.
    Agarwal P. Immunopathogenesis of Sjögren’s syndrome. J Indian Rheumatol Assoc. 2003;11:71–5.Google Scholar
  46. 46.
    Gono T, Kawaguchi Y, Katsumata Y, Takagi K, Tochimoto A, Baba S, et al. Clinical manifestations of neurological involvement in primary Sjögren’s syndrome. Clin Rheumatol. 2011;30(4):485–90.CrossRefPubMedGoogle Scholar
  47. 47.
    Park K, Haberberger RV, Gordon TP, Jackson MW. Antibodies interfering with the type 3 muscarinic receptor pathway inhibit gastrointestinal motility and cholinergic neurotransmission in Sjögren’s syndrome. Arthritis Rheum. 2011;63(5):1426–34.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2017

Authors and Affiliations

  1. 1.Department of NeurologyUniversity of Athens Medical School, Aeginition HospitalAthensGreece

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