Skip to main content
SpringerLink
Log in

Systemic Vasculitides

  • Chapter
  • First Online:
Arterial Disorders

  • 1342 Accesses

Abstract

Systemic vasculitis is defined as widespread vascular inflammation, which may be a primary condition or consequent to another pathologic process. These syndromes represent a wide and varied array of entities, the great majority of which are poorly understood in terms of their underlying pathogenic mechanisms and most appropriate and effective therapies. In this chapter, we will give a brief overview of the systemic vasculitides, excluding those directly caused by an infective agent. The vasculitides are a heterogenous group of diseases with poorly understood pathophysiology, making their classification a somewhat artificial and arbitrary attempt to segment entities along what is likely a continuous spectrum. The syndromes are discussed according to the predominant size of the vessels it affects using the recently revised Chapel Hill Consensus Conference Nomenclature of Vasculitides standards.

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 149.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
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 et al (2013) 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 65:1–11

    Article  CAS  PubMed  Google Scholar 

  2. Richards BL, March L, Gabriel SE (2010) Epidemiology of large-vessel vasculidities. Best Pract Res Clin Rheumatol 24:871–883

    Article  PubMed  Google Scholar 

  3. Kisza K, Murchison AP, Dai Y et al (2013) Giant cell arteritis incidence: analysis by season and year in mid-Atlantic United States. Clin Experiment Ophthalmol 41:577–581

    Article  PubMed  Google Scholar 

  4. Borchers AT, Gershwin ME (2012) Giant cell arteritis: a review of classification, pathophysiology, geoepidemiology and treatment. Autoimmun Rev 11:A544–A554

    Article  PubMed  Google Scholar 

  5. Robson JC, Kiran A, Maskell J et al (2015) The relative risk of aortic aneurysm in patients with giant cell arteritis compared with the general population of the UK. Ann Rheum Dis. 74:129–135

    Google Scholar 

  6. Salvarani C, Cantini F, Hunder GG (2008) Polymyalgia rheumatica and giant-cell arteritis. Lancet 372:234–245

    Article  PubMed  Google Scholar 

  7. Vaideeswar P, Deshpande JR (2013) Pathology of Takayasu arteritis: a brief review. Ann Pediatr Cardiol 6:52–58

    Article  PubMed Central  PubMed  Google Scholar 

  8. Keser G, Direskeneli H, Aksu K (2014) Management of Takayasu arteritis: a systematic review. Rheumatology (Oxford) 53:793–801

    Google Scholar 

  9. Colmegna I, Maldonado-Cocco JA (2005) Polyarteritis nodosa revisited. Curr Rheumatol Rep 7:288–296

    Article  PubMed  Google Scholar 

  10. Conn DL (1990) Polyarteritis. Rheum Dis Clin North Am 16:341–362

    CAS  PubMed  Google Scholar 

  11. Stanson AW, Friese JL, Johnson CM et al (2001) Polyarteritis nodosa: spectrum of angiographic findings. Radiographics 21:151–159

    Article  CAS  PubMed  Google Scholar 

  12. Guillevin L, Mahr A, Callard P et al (2005) Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore) 84:313–322

    Article  Google Scholar 

  13. Eleftheriou D, Dillon MJ, Tullus K et al (2013) Systemic polyarteritis nodosa in the young: a single-center experience over thirty-two years. Arthritis Rheum 65:2476–2485

    Article  CAS  PubMed  Google Scholar 

  14. De Menthon M, Mahr A (2011) Treating polyarteritis nodosa: current state of the art. Clin Exp Rheumatol 29:S110–S116

    PubMed  Google Scholar 

  15. Uehara R, Belay ED (2012) Epidemiology of Kawasaki disease in Asia, Europe, and the United States. J Epidemiol 22:79–85

    Article  PubMed Central  PubMed  Google Scholar 

  16. Newburger JW, Takahashi M, Gerber MA et al (2004) Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Pediatrics 114:1708–1733

    Article  PubMed  Google Scholar 

  17. Rowley AH (2011) Kawasaki disease: novel insights into etiology and genetic susceptibility. Annu Rev Med 62:69–77

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  18. Takahashi K, Oharaseki T, Yokouchi Y et al (2010) Kawasaki disease as a systemic vasculitis in childhood. Ann Vasc Dis 3:173–181

    Article  PubMed Central  PubMed  Google Scholar 

  19. Takahashi K, Oharaseki T, Yokouchi Y (2011) Pathogenesis of Kawasaki disease. Clin Exp Immunol 164(Suppl):20–22

    Article  PubMed Central  PubMed  Google Scholar 

  20. Ayusawa M, Sonobe T, Uemura S et al (2005) Revision of diagnostic guidelines for Kawasaki disease (the 5th revised edition). Pediatr Int 47:232–234

    Article  PubMed  Google Scholar 

  21. Furusho K, Kamiya T, Nakano H et al (1984) High-dose intravenous gammaglobulin for Kawasaki disease. Lancet 2:1055–1058

    Article  CAS  PubMed  Google Scholar 

  22. Luca NJC, Yeung RSM (2012) Epidemiology and management of Kawasaki disease. Drugs 72:1029–1038

    Article  CAS  PubMed  Google Scholar 

  23. Cotch MF et al (1996) The epidemiology of Wegener’s granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum 39:87–92

    Article  CAS  PubMed  Google Scholar 

  24. Wung PK, Stone JH (2006) Therapeutics of Wegener’s granulomatosis. Nat Clin Pract Rheumatol 2:192–200

    Article  CAS  PubMed  Google Scholar 

  25. Cabral DA, Uribe AG, Benseler S et al (2009) Classification, presentation, and initial treatment of Wegener’s granulomatosis in childhood. Arthritis Rheum 60:3413–3424

    Article  PubMed  Google Scholar 

  26. Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides. Arthritis Rheum 37:187–192

    Article  CAS  PubMed  Google Scholar 

  27. Riccieri V, Valesini G (2004) Treatment of Wegener’s granulomatosis. Reumatismo 56:69–76

    CAS  PubMed  Google Scholar 

  28. Mahr A (2001) Analysis of factors predictive of survival based on 49 patients with systemic Wegener’s granulomatosis and prospective follow-up. Rheumatology 40:492–498

    Article  CAS  PubMed  Google Scholar 

  29. Hoffman GS (1993) Wegener’s granulomatosis. Curr Opin Rheumatol 5:11–17

    Article  CAS  PubMed  Google Scholar 

  30. Dunogué B, Pagnoux C, Guillevin L (2011) Churg-Strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment. Semin Respir Crit Care Med 32:298–309

    Article  PubMed  Google Scholar 

  31. Noth I, Strek ME, Leff AR (2003) Churg-Strauss syndrome. Lancet 361:587–594

    Article  PubMed  Google Scholar 

  32. Vaglio A, Buzio C, Zwerina J (2013) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): state of the art. Allergy 68:261–273

    Article  CAS  PubMed  Google Scholar 

  33. Mahr A, Moosig F, Neumann T et al (2014) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): evolutions in classification, etiopathogenesis, assessment and management. Curr Opin Rheumatol 26:16–23

    Article  CAS  PubMed  Google Scholar 

  34. Comarmond C, Pagnoux C, Khellaf M et al (2013) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term follow up of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum 65:270–281

    Article  PubMed  Google Scholar 

  35. Lanham JG, Elkon KB, Pusey CD, Hughes GR (1984) Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Med (Baltimore) 63:65–81

    Article  CAS  Google Scholar 

  36. Samson M, Puechal X, Devilliers H et al (2013) Long-term outcomes of 118 patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) enrolled in two prospective trials. J Autoimmun 43:60–69

    Article  PubMed  Google Scholar 

  37. Mohammad AJ, Jacobsson LTH, Mahr AD et al (2007) Prevalence of Wegener’s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford) 46:1329–1337

    Article  CAS  Google Scholar 

  38. Chung SA, Seo P (2010) Microscopic polyangiitis. Rheum Dis Clin North Am 36:545–558

    Article  PubMed Central  PubMed  Google Scholar 

  39. Guillevin L, Durand-Gasselin B, Cevallos R et al (1999) Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 42:421–430

    Article  CAS  PubMed  Google Scholar 

  40. Kawakami T, Soma Y, Saito C et al (2006) Cutaneous manifestations in patients with microscopic polyangiitis: two case reports and a minireview. Acta Derm Venereol 86:144–147

    PubMed  Google Scholar 

  41. Agard C, Mouthon L, Mahr A, Guillevin L (2003) Microscopic polyangiitis and polyarteritis nodosa: how and when do they start? Arthritis Rheum 49:709–715

    Article  PubMed  Google Scholar 

  42. Salama AD, Levy JB, Lightstone L, Pusey CD (2001) Goodpasture’s disease. Lancet 358:917–920

    Article  CAS  PubMed  Google Scholar 

  43. Chan AL, Louie S, Leslie KO et al (2011) Cutting edge issues in Goodpasture’s disease. Clin Rev Allergy Immunol 41:151–162

    Article  CAS  PubMed  Google Scholar 

  44. Hellmark T, Segelmark M (2014) Diagnosis and classification of Goodpasture’s disease (anti-GBM). J Autoimmun 48–49:108–112

    Google Scholar 

  45. Syeda UA, Singer NG, Magrey M (2013) Anti-glomerular basement membrane antibody disease treated with rituximab: a case-based review. Semin Arthritis Rheum 42:567–572

    Article  PubMed  Google Scholar 

  46. Levin M, Rigden SP, Pincott JR et al (1983) Goodpasture’s syndrome: treatment with plasmapheresis, immunosuppression, and anticoagulation. Arch Dis Child 58:697–702

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  47. Gardner-Medwin JMM, Dolezalova P, Cummins C, Southwood TR (2002) Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 360:1197–1202

    Article  PubMed  Google Scholar 

  48. Yang Y-H, Yu H-H, Chiang B-L (2014) The diagnosis and classification of Henoch-Schönlein purpura: an updated review. Autoimmun Rev. doi:10.1016/j.autrev.2014.01.031

    Google Scholar 

  49. Linskey KR, Kroshinsky D, Mihm MC, Hoang MP (2012) Immunoglobulin-A–associated small-vessel vasculitis: a 10-year experience at the Massachusetts General Hospital. J Am Acad Dermatol 66:813–822

    Article  PubMed  Google Scholar 

  50. Saulsbury FT (1999) Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Med (Baltimore) 78:395–409

    Article  CAS  Google Scholar 

  51. Pillebout E, Thervet E, Hill G et al (2002) Henoch-Schönlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol 13:1271–1278

    Article  PubMed  Google Scholar 

  52. Chartapisak W, Opastirakul S, Hodson EM et al (2009) Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP). Cochrane database Syst Rev (3):CD005128. doi:10.1002/14651858.CD005128.pub2

  53. Trnka P (2013) Henoch-Schönlein purpura in children. J Paediatr Child Health 49:995–1003

    Article  PubMed  Google Scholar 

  54. Trapani S, Micheli A, Grisolia F et al (2005) Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum 35:143–153

    Google Scholar 

  55. Bayrakci US, Topaloglu R, Soylemezoglu O et al (2007) Effect of early corticosteroid therapy on development of Henoch-Schönlein nephritis. J Nephrol 20:406–409

    CAS  PubMed  Google Scholar 

  56. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R et al (2004) Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol 138:373–380

    Article  PubMed  Google Scholar 

  57. International Study Group for Behçet’s Disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335:1078–1080

    Google Scholar 

  58. O’Neill TW, Rigby AS, Silman AJ, Barnes C (1994) Validation of the International Study Group criteria for Behçet’s disease. Br J Rheumatol 33:115–117

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Pathology, Intermountain Medical Center/University of Utah, Electron Microscopy Lab, Intermountain Central Lab, 5252 S Intermountain Dr., Murray, UT, 84157, USA

    Dylan V. Miller MD

  2. Internal Medicine, Intermountain Medical Center, 5121 S Cottonwood Street, Murray, UT, 84157, USA

    C. Taylor Duncan MD

Authors
  1. Dylan V. Miller MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. C. Taylor Duncan MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Dylan V. Miller MD .

Editor information

Editors and Affiliations

  1. American University of Beirut Medical Center, Beirut, Lebanon

    Adel Berbari

  2. University of Milano Bicocca IRCCS Istituto Auxologico Italiano, Milan, Italy

    Giuseppe Mancia

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Miller, D.V., Duncan, C.T. (2015). Systemic Vasculitides. In: Berbari, A., Mancia, G. (eds) Arterial Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-14556-3_18

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-14556-3_18

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-14555-6

  • Online ISBN: 978-3-319-14556-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation