Skip to main content
SpringerLink
Log in

Pathophysiology and Principles of Management of Vasculitis and Fibromuscular Dysplasia

  • Chapter
  • First Online:
Mechanisms of Vascular Disease

  • 1045 Accesses

Abstract

Vasculitis is caused by immune-mediated inflammation of blood vessel walls, resulting in tissue ischaemia, aneurysm formation, dissection and/or rupture. Diverse clinical presentations relate to distinct pathology and pathological mechanisms, size of affected vessels, pattern of organ and tissue involvement, and associated systemic inflammatory disease. The aetiology of most types of vasculitis is unknown, and all ages may be affected. Treatment is directed to known causes such as viruses and drugs, suppression of inflammation using corticosteroids and other immune suppressants, and, where indicated, revascularisation. Early recognition and institution of treatment enables prevention of permanent organ damage and has led to marked improvement in patient outcomes, with reduced mortality. Diagnosis is usually confirmed by biopsy of affected tissues, or imaging modalities when this is not practical. Adjunctive investigations of blood and urine are important to define specific subtypes of vasculitis, in order to determine the most appropriate treatment. Prevention and management of treatment side-effects including infection, osteoporosis, peptic ulceration, diabetes, hypertension, dyslipidaemia, and mood disorders, should be incorporated into routine care. Technological advances and collaborative research efforts underpin evolving concepts in the pathophysiology and principles of management of vasculitis.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. 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. https://doi.org/10.1002/art.37715.

    Article  CAS  PubMed  Google Scholar 

  2. Al-Mousawi AZ, Gurney SP, Lorenzi AR, Pohl U, Dayan M, Mollan SP. Reviewing the pathophysiology behind the advances in the management of giant cell arteritis. Ophthalmol Ther. 2019;8:177–93. https://doi.org/10.1007/s40123-019-0171-0.

    Article  PubMed  PubMed Central  Google Scholar 

  3. Weyand CM, Watanabe R, Zhang H, Akiyama M, Berry GJ, Goronzy JJ. Cytokines, growth factors and proteases in medium and large vessel vasculitis. Clin Immunol. 2019;206:33–41. https://doi.org/10.1016/j.clim.2019.02.007.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Watanabe R, Zhang H, Berry G, Goronzy JJ, Weyand CM. Immune checkpoint dysfunction in medium and large vessel Vasculitis. Am J Physiol Heart Circ Physiol. 2017;312:H1052–9. https://doi.org/10.1152/ajpheart.00024.2017.

    Article  PubMed  PubMed Central  Google Scholar 

  5. Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum. 1990;33:1122–8. https://doi.org/10.1002/art.1780330810.

    Article  CAS  PubMed  Google Scholar 

  6. Chung SH, Morcos MB, Ng B. Determinants of positive temporal artery biopsies in the veterans health administration national database cohort. Arthritis Care Res. 2019. https://doi.org/10.1002/acr.23897. [Epub ahead of print].

  7. Dejaco C, Ramiro S, Duftner C, Besson FL, Bley TA, Blockmans D, et al. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice. Ann Rheum Dis. 2018;77:636–43. https://doi.org/10.1136/annrheumdis-2017-212649.

    Article  PubMed  Google Scholar 

  8. Sammel AM, Hsiao E, Schembri G, Nguyen K, Brewer J, Schrieber L, et al. Diagnostic accuracy of positron emission tomography/computed tomography of the head, neck, and chest for giant cell arteritis: a prospective, double-blind, cross-sectional study. Arthritis Rheumatol. 2019;71:1319–28. https://doi.org/10.1002/art.40864.

    Article  PubMed  Google Scholar 

  9. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2009;68:318–23. https://doi.org/10.1136/ard.2008.088351.

    Article  CAS  PubMed  Google Scholar 

  10. Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M, Blockmans D, et al. Glucocorticoid doses and acute-phase reactants at giant cell arteritis flare in a randomized trial of tocilizumab. Arthritis Rheumatol. 2019;71:1329–38. https://doi.org/10.1002/art.40876.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Adler S, Reichenbach S, Gloor A, Yerly D, Cullmann JL, Villiger PM. Risk of relapse after discontinuation of tocilizumab therapy in giant cell arteritis. Rheumatology (Oxford). 2019;58:1639–43. https://doi.org/10.1093/rheumatology/kez091.

    Article  Google Scholar 

  12. Unizony S, Stone JH, Stone JR. New treatment strategies in large-vessel vasculitis. Curr Opin Rheumatol. 2013;25:3–9. https://doi.org/10.1097/BOR.0b013e32835b133a.

    Article  CAS  PubMed  Google Scholar 

  13. Watts RA. Evolving concepts in classification of systemic vasculitis: where are we and what is the way forward? Int J Rheum Dis. 2019;22(Suppl 1):21–7. https://doi.org/10.1111/1756-185X.13304.

    Article  PubMed  Google Scholar 

  14. Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990;33:1129–34. https://doi.org/10.1002/art.1780330811.

    Article  CAS  PubMed  Google Scholar 

  15. Pazzola G, Muratore F, Pipitone N, Crescentini F, Cacoub P, Boiardi L, et al. Rituximab therapy for Takayasu arteritis: a seven patients experience and a review of the literature. Rheumatology (Oxford). 2017;57:1151–5. https://doi.org/10.1093/rheumatology/kex249. [Epub ahead of print].

    Article  CAS  Google Scholar 

  16. Ozen S. The changing face of polyarteritis nodosa and necrotizing vasculitis. Nat Rev Rheumatol. 2017;13:381–6. https://doi.org/10.1038/nrrheum.2017.68.

    Article  PubMed  Google Scholar 

  17. Dietz SM, van Stijn D, Burgner D, Levin M, Kuipers IM, Hutten BA, et al. Dissecting Kawasaki disease: a state-of-the-art review. Eur J Pediatr. 2017;176:995–1009. https://doi.org/10.1007/s00431-017-2937-5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. McCrindle BW, Rowley AH, Newburger JW, Burns JC, Bolger AF, Gewitz M, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a scientific statement for health professionals from the American Heart Association. Circulation. 2017;135:e927–99. https://doi.org/10.1161/CIR.0000000000000484.

    Article  PubMed  Google Scholar 

  19. Coit P, Direskeneli H, Sawalha AH. An update on the role of epigenetics in systemic vasculitis. Curr Opin Rheumatol. 2018;30:4–15. https://doi.org/10.1097/BOR.0000000000000451.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Al-Hussain T, Hussein MH, Conca W, Al Mana H, Akhtar M. Pathophysiology of ANCA-associated vasculitis. Adv Anat Pathol. 2017;24:226–34. https://doi.org/10.1097/PAP.0000000000000154.

    Article  PubMed  Google Scholar 

  21. Lopalco G, Rigante D, Venerito V, Emmi G, Anelli MG, Lapadula G, et al. Management of small vessel vasculitides. Curr Rheumatol Rep. 2016;18:36. https://doi.org/10.1007/s11926-016-0580-1.

    Article  CAS  PubMed  Google Scholar 

  22. Sharma A, Dogra S, Sharma K. Granulomatous vasculitis. Dermatol Clin. 2015;33:475–87. https://doi.org/10.1016/j.det.2015.03.012.

    Article  CAS  PubMed  Google Scholar 

  23. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Arthritis Rheum. 1994;37:187–92. https://doi.org/10.1002/art.1780370206.

    Article  CAS  PubMed  Google Scholar 

  24. Jones RB, Hiemstra TF, Ballarin J, Blockmans DE, Brogan P, Bruchfeld A, et al. Mycophenolate mofetil versus cyclophosphamide for remission induction in ANCA-associated vasculitis: a randomised, non-inferiority trial. Ann Rheum Dis. 2019;78:399–405. https://doi.org/10.1136/annrheumdis-2018-214245.

    Article  CAS  PubMed  Google Scholar 

  25. Walsh M, Merkel PA, Peh C-A, Szpirt WM, Puechal X, Fujimoto S, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med. 2020;382:622–31. https://doi.org/10.1056/NEJMoa1803537.

  26. Faverio P, Bonaiti G, Bini F, Vaghi A, Pesci A. Mepolizumab as the first targeted treatment for eosinophilic granulomatosis with polyangiitis: a review of current evidence and potential place in therapy. Ther Clin Risk Manag. 2018;14:2385–96. https://doi.org/10.2147/TCRM.S159949.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Hetland LE, Susrud KS, Lindahl KH, Bygum A. Henoch-Schonlein purpura: a literature review. Acta Derm Venereol. 2017;97:1160–6. https://doi.org/10.2340/00015555-2733.

    Article  CAS  PubMed  Google Scholar 

  28. Lopez-Mejias R, Castaneda S, Genre F, Remuzgo-Martinez S, Carmona FD, Llorca J, et al. Genetics of immunoglobulin-A vasculitis (Henoch-Schonlein purpura): an updated review. Autoimmun Rev. 2018;17:301–15. https://doi.org/10.1016/j.autrev.2017.11.024.

    Article  CAS  PubMed  Google Scholar 

  29. Belizna CC, Hamidou MA, Levesque H, Guillevin L, Shoenfeld Y. Infection and vasculitis. Rheumatology (Oxford). 2009;48:475–82. https://doi.org/10.1093/rheumatology/kep026.

    Article  PubMed Central  Google Scholar 

  30. Emmi G, Bettiol A, Silvestri E, Di Scala G, Becatti M, Fiorillo C, et al. Vascular Behcet’s syndrome: an update. Intern Emerg Med. 2018;176:995–1009. https://doi.org/10.1007/s11739-018-1991-y.

    Article  Google Scholar 

  31. Emmi G, Silvestri E, Squatrito D, D’Elios MM, Ciucciarelli L, Prisco D, et al. Behçet’s syndrome pathophysiology and potential therapeutic targets. Intern Emerg Med. 2014;9:257–65. https://doi.org/10.1007/s11739-013-1036-5.

    Article  PubMed  Google Scholar 

  32. Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77:808–18. https://doi.org/10.1136/annrheumdis-2018-213225.

    Article  PubMed  Google Scholar 

  33. Retamozo S, Gheitasi H, Quartuccio L, Kostov B, Corazza L, Bové A, et al. Cryoglobulinaemic vasculitis at diagnosis predicts mortality in primary Sjögren syndrome: analysis of 515 patients. Rheumatology (Oxford). 2016;55:1443–51. https://doi.org/10.1093/rheumatology/kew194.

    Article  Google Scholar 

  34. Bledsoe JR, Della-Torre E, Rovati L, Deshpande V. IgG4-related disease: review of the histopathologic features, differential diagnosis, and therapeutic approach. APMIS. 2018;126:459–76. https://doi.org/10.1111/apm.12845.

    Article  CAS  PubMed  Google Scholar 

  35. Wallace ZS, Zhang Y, Perugino CA, Naden R, Choi HK, Stone JH. Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts. Ann Rheum Dis. 2019;78:406–12. https://doi.org/10.1136/annrheumdis-2018-214603.

    Article  CAS  PubMed  Google Scholar 

  36. Mattoo H, Mahajan VS, Maehara T, Deshpande V, Della-Torre E, Wallace ZS, et al. Clonal expansion of CD4+ cytotoxic T lymphocytes in patients with IgG4-related disease. J Allergy Clin Immunol. 2016;138:825–38. https://doi.org/10.1016/j.jaci.2015.12.1330.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. Mattoo H, Stone JH, Pillai S. Clonally expanded cytotoxic CD4+ T cells and the pathogenesis of IgG4-related disease. Autoimmunity. 2017;50:19–24. https://doi.org/10.1080/08916934.2017.1280029.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  38. Choi HK, Merkel PA, Walker AM, Niles JL. Drug-associated antineutrophil cytoplasmic antibody–positive vasculitis: prevalence among patients with high titers of antimyeloperoxidase antibodies. Arthritis Rheum. 2000;43:405–13. https://doi.org/10.1002/1529-0131(200002)43:2<405::aid-anr22>3.0.co;2-5.

    Article  CAS  PubMed  Google Scholar 

  39. Hacking S, Uppal NN, Khan N, Ionescu M, Bijol V. Systemic p-ANCA vasculitis with fatal outcome, arising in the setting of methimazole use. Clin Nephrol Case Stud. 2019;7:23–6. https://doi.org/10.5414/CNCS109759.

    Article  PubMed  PubMed Central  Google Scholar 

  40. Radic M, Martinovic Kaliterna D, Radic J. Drug-induced vasculitis: a clinical and pathological review. Neth J Med. 2012;70:12–7.

    CAS  PubMed  Google Scholar 

  41. Calabrese LH, Calabrese C, Cappelli LC. Rheumatic immune-related adverse events from cancer immunotherapy. Nat Rev Rheumatol. 2018;14:569–79. https://doi.org/10.1038/s41584-018-0074-9.

    Article  PubMed  Google Scholar 

  42. Wigley FM, Flavahan NA. Raynaud’s phenomenon. N Engl J Med. 2016;375:556–65. https://doi.org/10.1056/NEJMra1507638.

    Article  CAS  PubMed  Google Scholar 

  43. Weinberg I, Weinberg MD. Non-atherosclerotic arterial disorders of the lower extremities. panvascular medicine. Berlin: Springer; 2015. p. 3007–30.

    Google Scholar 

  44. Dellalibera-Joviliano R, Joviliano EE, Silva JS, Evora PRB. Activation of cytokines corroborate with development of inflammation and autoimmunity in thromboangiitis obliterans patients. Clin Exp Immunol. 2012;170:28–35. https://doi.org/10.1111/j.1365-2249.2012.04624.x.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  45. Akar AR, İnan MB, Baran Ç. Thromboangiitis obliterans. Curr Treat Options Rheumatol. 2016;2:178–95. https://doi.org/10.1007/s40674-016-0047-6.

    Article  Google Scholar 

  46. Narvaez J, Garcia-Gomez C, Alvarez L, Santo P, Aparicio M, Pascual M, et al. Efficacy of bosentan in patients with refractory thromboangiitis obliterans (Buerger disease): a case series and review of the literature. Medicine (Baltimore). 2016;95:e5511. https://doi.org/10.1097/MD.0000000000005511.

    Article  PubMed Central  Google Scholar 

  47. Lee CY, Choi K, Kwon H, Ko G-Y, Han Y, Kwon T-W, et al. Outcomes of endovascular treatment versus bypass surgery for critical limb ischemia in patients with thromboangiitis obliterans. PLoS One. 2018;13:e0205305. https://doi.org/10.1371/journal.pone.0205305.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Gornik HL, Persu A, Adlam D, Aparicio LS, Azizi M, Boulanger M, et al. First international consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med. 2019;24:164–89. https://doi.org/10.1177/1358863x18821816.

    Article  PubMed  Google Scholar 

  49. Kadian-Dodov D, Gornik HL, Gu X, Froehlich J, Bacharach JM, Chi YW, et al. Dissection and aneurysm in patients with fibromuscular dysplasia: findings from the U.S. registry for FMD. J Am Coll Cardiol. 2016;68:176–85. https://doi.org/10.1016/j.jacc.2016.04.044.

    Article  PubMed  Google Scholar 

  50. Plouin PF, Baguet JP, Thony F, Ormezzano O, Azarine A, Silhol F, et al. High prevalence of multiple arterial bed lesions in patients with fibromuscular dysplasia: the ARCADIA registry (assessment of renal and cervical artery dysplasia). Hypertension. 2017;70:652–8. https://doi.org/10.1161/hypertensionaha.117.09539.

    Article  CAS  PubMed  Google Scholar 

Further Reading

Download references

Author information

Authors and Affiliations

  1. Rheumatology Department, The Queen Elizabeth Hospital, Woodville, SA, Australia

    Maureen Rischmueller & Sarah Downie-Doyle

  2. Rheumatology Department, Royal Darwin Hospital, Darwin, NT, Australia

    Maureen Rischmueller

  3. Discipline of Medicine, The University of Adelaide, Adelaide, SA, Australia

    Maureen Rischmueller

  4. Discipline of Surgery, The Queen Elizabeth Hospital, The University of Adelaide, Woodville, SA, Australia

    Robert Fitridge

Authors
  1. Maureen Rischmueller
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sarah Downie-Doyle
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Robert Fitridge
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Maureen Rischmueller .

Editor information

Editors and Affiliations

  1. Discipline of Surgery, The University of Adelaide, Adelaide, SA, Australia

    Robert Fitridge

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Rischmueller, M., Downie-Doyle, S., Fitridge, R. (2020). Pathophysiology and Principles of Management of Vasculitis and Fibromuscular Dysplasia. In: Fitridge, R. (eds) Mechanisms of Vascular Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-43683-4_16

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-43683-4_16

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-43682-7

  • Online ISBN: 978-3-030-43683-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation