Advertisement

Vascular Disorders: Vasculitis

  • Serge Weis
  • Michael Sonnberger
  • Andreas Dunzinger
  • Eva Voglmayr
  • Martin Aichholzer
  • Raimund Kleiser
  • Peter Strasser
Chapter
  • 414 Downloads

Abstract

Vasculitis is an inflammation of blood vessel walls due to infectious or non-infectious reasons. The size of the vessel serves for disease classification (e.g., large vessel vasculitis, medium vessel vasculitis, small vessel vasculitis). The morphological changes of the involved vessels might be variable. The pathogenesis of non-infectious vasculitis might be autoimmune, associated with an immunologic reaction directed at elastin, associated with human leukocyte antigen (HLA)-B∗51 (Behçet’s disease), associated with immune and inflammatory response, antineutrophil cytoplasmic antibody-associated vasculitides. Germ line mutations lead to adenosine deaminase 2 deficiency.

Treatment in infectious vasculitis uses antiviral and antibacterial agents, while in non-infectious vasculitis corticosteroids and immune suppressants (cyclophosphamide, methotrexate, azathioprine) are used. Clinical outcome depends on the involvement of other organs and pre-existing illnesses.

Selected References

  1. Adams HP Jr (2014) Cerebral vasculitis. Handb Clin Neurol 119:475–494.  https://doi.org/10.1016/b978-0-7020-4086-3.00031-xGoogle Scholar
  2. Brogan P, Eleftheriou D (2018) Vasculitis update: pathogenesis and biomarkers. Pediatr Nephrol (Berlin, Germany) 33(2):187–198.  https://doi.org/10.1007/s00467-017-3597-4Google Scholar
  3. Bucerius J (2016) Monitoring vasculitis with 18F-FDG PET. Q J Nucl Med Mol Imaging 60(3):219–235Google Scholar
  4. Calabrese LH, Mallek JA (1988) Primary angiitis of the central nervous system. Report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine 67(1):20–39Google Scholar
  5. Carmona FD, Martin J, Gonzalez-Gay MA (2015) Genetics of vasculitis. Curr Opin Rheumatol 27(1):10–17.  https://doi.org/10.1097/bor.0000000000000124Google Scholar
  6. Dutra LA, de Souza AW, Grinberg-Dias G, Barsottini OG, Appenzeller S (2017) Central nervous system vasculitis in adults: an update. Autoimmun Rev 16(2):123–131.  https://doi.org/10.1016/j.autrev.2016.12.001Google Scholar
  7. Elefante E, Monti S, Bond M, Lepri G, Quartuccio L, Talarico R, Baldini C (2017) One year in review 2017: systemic vasculitis. Clin Exp Rheumatol 35 Suppl 103(1):5–26Google Scholar
  8. Giannini C, Salvarani C, Hunder G, Brown RD (2012) Primary central nervous system vasculitis: pathology and mechanisms. Acta Neuropathol 123(6):759–772.  https://doi.org/10.1007/s00401-012-0973-9Google Scholar
  9. Gutierrez-Gonzalez LA (2016) Biological therapy-induced systemic vasculitis. Curr Rheumatol Rep 18(7):39.  https://doi.org/10.1007/s11926-016-0588-6Google Scholar
  10. Hajj-Ali RA, Calabrese LH (2014) Diagnosis and classification of central nervous system vasculitis. J Autoimmun 48–49:149–152.  https://doi.org/10.1016/j.jaut.2014.01.007Google Scholar
  11. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA (2013) 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65(1):1–11.  https://doi.org/10.1002/art.37715Google Scholar
  12. John S, Hajj-Ali RA (2014) CNS vasculitis. Semin Neurol 34(4):405–412.  https://doi.org/10.1055/s-0034-1390389Google Scholar
  13. Koster MJ, Warrington KJ (2016) Recent advances in understanding and treating vasculitis. F1000Res 5.  https://doi.org/10.12688/f1000research.8403.1
  14. Legendre P, Regent A, Thiebault M, Mouthon L (2017) Anti-endothelial cell antibodies in vasculitis: a systematic review. Autoimmun Rev 16(2):146–153.  https://doi.org/10.1016/j.autrev.2016.12.012Google Scholar
  15. Loricera J, Blanco R, Hernandez JL, Martinez-Rodriguez I, Carril JM, Lavado C, Jimenez M, Gonzalez-Vela C, Gonzalez-Gay MA (2015) Use of positron emission tomography (PET) for the diagnosis of large-vessel vasculitis. Rev Esp Med Nucl Imagen Mol 34(6):372–377.  https://doi.org/10.1016/j.remn.2015.07.002Google Scholar
  16. Misra DP, Agarwal V (2016) Innate immune cells in the pathogenesis of primary systemic vasculitis. Rheumatol Int 36(2):169–182.  https://doi.org/10.1007/s00296-015-3367-1Google Scholar
  17. Mossa-Basha M, Alexander M, Gaddikeri S (2016) Vessel wall imaging for intracranial vascular disease evaluation. J Neurointerv Surg 8(11):1154–1159.  https://doi.org/10.1136/neurintsurg-2015-012127Google Scholar
  18. Muratore F, Pipitone N, Salvarani C, Schmidt WA (2016) Imaging of vasculitis: state of the art. Best Pract Res Clin Rheumatol 30(4):688–706.  https://doi.org/10.1016/j.berh.2016.09.010Google Scholar
  19. Nocton JJ (2017) Usual and unusual manifestations of systemic and central nervous system vasculitis. Pediatr Clin N Am 64(1):185–204.  https://doi.org/10.1016/j.pcl.2016.08.013Google Scholar
  20. Peleg H, Ben-Chetrit E (2017) Vasculitis in the autoinflammatory diseases. Curr Opin Rheumatol 29(1):4–11.  https://doi.org/10.1097/bor.0000000000000347Google Scholar
  21. Prieto-Gonzalez S, Arguis P, Cid MC (2015) Imaging in systemic vasculitis. Curr Opin Rheumatol 27(1):53–62.  https://doi.org/10.1097/bor.0000000000000130Google Scholar
  22. Prieto-Gonzalez S, Espigol-Frigole G, Garcia-Martinez A, Alba MA, Tavera-Bahillo I, Hernandez-Rodriguez J, Renu A, Gilabert R, Lomena F, Cid MC (2016) The expanding role of imaging in systemic vasculitis. Rheum Dis Clin N Am 42(4):733–751.  https://doi.org/10.1016/j.rdc.2016.07.009Google Scholar
  23. Renauer P, Coit P, Sawalha AH (2016) Epigenetics and vasculitis: a comprehensive review. Clin Rev Allergy Immunol 50(3):357–366.  https://doi.org/10.1007/s12016-015-8495-6Google Scholar
  24. Salvarani C, Brown RD Jr, Hunder GG (2017) Adult primary central nervous system vasculitis. Israel Med Assoc J 19(7):448–453Google Scholar
  25. Shirai T, Hilhorst M, Harrison DG, Goronzy JJ, Weyand CM (2015) Macrophages in vascular inflammation—from atherosclerosis to vasculitis. Autoimmunity 48(3):139–151.  https://doi.org/10.3109/08916934.2015.1027815Google Scholar
  26. Silva de Souza AW (2015) Autoantibodies in systemic vasculitis. Front Immunol 6:184.  https://doi.org/10.3389/fimmu.2015.00184Google Scholar
  27. Teng GG, Chatham WW (2015) Vasculitis related to viral and other microbial agents. Best Pract Res Clin Rheumatol 29(2):226–243.  https://doi.org/10.1016/j.berh.2015.05.007Google Scholar
  28. Thomas K, Vassilopoulos D (2017) Infections and vasculitis. Curr Opin Rheumatol 29(1):17–23.  https://doi.org/10.1097/bor.0000000000000348Google Scholar
  29. Twilt M, Benseler SM (2016) Central nervous system vasculitis in adults and children. Handb Clin Neurol 133:283–300.  https://doi.org/10.1016/b978-0-444-63432-0.00016-5Google Scholar
  30. Watanabe R, Zhang H, Berry G, Goronzy JJ, Weyand CM (2017) Immune checkpoint dysfunction in large and medium vessel vasculitis. Am J Phys Heart Circ Phys 312(5):H1052–h1059.  https://doi.org/10.1152/ajpheart.00024.2017Google Scholar
  31. Yates M, Watts R (2017) ANCA-associated vasculitis. Clin Med (Lond) 17(1):60–64.  https://doi.org/10.7861/clinmedicine.17-1-60Google Scholar

Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2019

Authors and Affiliations

  • Serge Weis
    • 1
  • Michael Sonnberger
    • 2
  • Andreas Dunzinger
    • 3
  • Eva Voglmayr
    • 2
  • Martin Aichholzer
    • 4
  • Raimund Kleiser
    • 2
  • Peter Strasser
    • 5
  1. 1.Division of Neuropathology, Neuromed CampusKepler University Hospital, Johannes Kepler UniversityLinzAustria
  2. 2.Department of Neuroradiology, Neuromed CampusKepler University Hospital, Johannes Kepler UniversityLinzAustria
  3. 3.Department of Neuro-Nuclear Medicine, Neuromed CampusKepler University Hospital, Johannes Kepler UniversityLinzAustria
  4. 4.Department of Neurosurgery, Neuromed CampusKepler University Hospital, Johannes Kepler UniversityLinzAustria
  5. 5.PMU University Institute for Medical & Chemical Laboratory DiagnosticsSalzburgAustria

Personalised recommendations