Skip to main content
SpringerLink
Log in

Vasculitis

  • Chapter
  • First Online:
Comorbidity in Rheumatic Diseases

  • 1111 Accesses

Abstract

Vasculitis is a heterogeneous group of disorders characterized by inflammation of the wall of blood vessels of various sizes, including veins, arteries, and capillaries. Comorbidities may occur as an intrinsic component of systemic vasculitis or as adverse effects of treatment. This chapter describes the various comorbidities associated with vasculitis, with emphasis on atherosclerotic cardiovascular disease, treatment-associated infections, thrombosis, and malignancy. Careful and meticulous assessment of comorbidities is mandatory at the time of diagnosis of the various vasculitides as well as during the active phase and at remission.

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 109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 139.99
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):1–11.

    Article  CAS  PubMed  Google Scholar 

  2. Matteson EL, Buttgereit F, Dejaco C, Dasgupta B. Glucocorticoids for management of polymyalgia rheumatica and giant cell arteritis. Rheum Dis Clin N Am. 2016;42(1):75–90.

    Article  Google Scholar 

  3. de Souza AW, de Carvalho JF. Diagnostic and classification criteria of Takayasu arteritis. J Autoimmun. 2014;48-49:79–83.

    Article  PubMed  CAS  Google Scholar 

  4. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. European Vasculitis Study Group. EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2009;68(3):318–23.

    Article  CAS  PubMed  Google Scholar 

  5. Ferfar Y, Mirault T, Desbois AC, Comarmond C, Messas E, Savey L, et al. Biotherapies in large vessel vasculitis. Autoimmun Rev. 2016;15(6):544–51.

    Article  CAS  PubMed  Google Scholar 

  6. Hollan I, Meroni PL, Ahearn JM, Cohen Tervaert JW, Curran S, Goodyear CS, et al. Cardiovascular disease in autoimmune rheumatic diseases. Autoimmun Rev. 2013;12(10):1004–15.

    Article  CAS  PubMed  Google Scholar 

  7. Mason JC, Libby P. Cardiovascular disease in patients with chronic inflammation: mechanisms underlying premature cardiovascular events in rheumatologic conditions. Eur Heart J. 2015;36(8):482–9c.

    Article  PubMed  Google Scholar 

  8. Ninan J, Nguyen AM, Cole A, Rischmueller M, Dodd T, Roberts-Thomson P, et al. Mortality in patients with biopsy-proven giant cell arteritis: a south Australian population-based study. J Rheumatol. 2011;38(10):2215–7.

    Article  PubMed  Google Scholar 

  9. Baslund B, Helleberg M, Faurschou M, Obel N. Mortality in patients with giant cell arteritis. Rheumatology (Oxford). 2015;54(1):139–43.

    Article  Google Scholar 

  10. Tomasson G, Peloquin C, Mohammad A, Love TJ, Zhang Y, Choi HK, et al. Risk for cardiovascular disease early and late after a diagnosis of giant-cell arteritis: a cohort study. Ann Intern Med. 2014;160(2):73–80.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Gonzalez-Gay MA, Vazquez-Rodriguez TR, Gomez-Acebo I, Pego-Reigosa R, Lopez-Diaz MJ, Vazquez-Triñanes MC, et al. Strokes at time of disease diagnosis in a series of 287 patients with biopsy-proven giant cell arteritis. Medicine (Baltimore). 2009;88(4):227–35.

    Article  Google Scholar 

  12. Samson M, Jacquin A, Audia S, Daubail B, Devilliers H, Petrella T, et al. Stroke associated with giant cell arteritis: a population-based study. J Neurol Neurosurg Psychiatry. 2015;86(2):216–21.

    Article  PubMed  Google Scholar 

  13. Amiri N, De Vera M, Choi HK, Sayre EC, Avina-Zubieta JA. Increased risk of cardiovascular disease in giant cell arteritis: a general population-based study. Rheumatology (Oxford). 2016;55(1):33–40.

    Article  Google Scholar 

  14. González-Gay MA, Blanco R, Abraira V, García-Porrúa C, Ibáñez D, García-Pais MJ, et al. Giant cell arteritis in Lugo, Spain, is associated with low long-term mortality. J Rheumatol. 1997;24(11):2171–6.

    PubMed  Google Scholar 

  15. Udayakumar PD, Chandran AK, Crowson CS, Warrington KJ, Matteson EL. Cardiovascular risk and acute coronary syndrome in giant cell arteritis: a population-based retrospective cohort study. Arthritis Care Res (Hoboken). 2015;67(3):396–402.

    Article  Google Scholar 

  16. Petri H, Nevitt A, Sarsour K, Napalkov P, Collinson N. Incidence of giant cell arteritis and characteristics of patients: data-driven analysis of comorbidities. Arthritis Care Res (Hoboken). 2015;67(3):390–5.

    Article  CAS  Google Scholar 

  17. Filer A, Nicholls D, Corston R, Carey P, Bacon P. Takayasu arteritis and atherosclerosis: illustrating the consequences of endothelial damage. J Rheumatol. 2001;28(12):2752–3.

    CAS  PubMed  Google Scholar 

  18. Numano F, Okawara M, Inomata H, Kobayashi Y. Takayasu’s arteritis. Lancet. 2000;356:1023–5.

    Article  CAS  PubMed  Google Scholar 

  19. Seyahi E, Ugurlu S, Cumali R, Balci H, Seyahi N, Yurdakul S, et al. Atherosclerosis in Takayasu arteritis. Ann Rheum Dis. 2006;65(9):1202–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Duarte MM, Geraldes R, Sousa R, Alarcão J, Costa J. Stroke and transient ischemic attack in Takayasu’s arteritis: a systematic review and meta-analysis. J Stroke Cerebrovasc Dis. 2016;25(4):781–91.

    Article  PubMed  Google Scholar 

  21. Rav-Acha M, Plot L, Peled N, Amital H. Coronary involvement in Takayasu’s arteritis. Autoimmun Rev. 2007;6(8):566–71.

    Article  PubMed  Google Scholar 

  22. Bossert M, Prati C, Balblanc JC, Lohse A, Wendling D. Aortic involvement in giant cell arteritis: current data. Joint Bone Spine. 2011;78(3):246–51.

    Article  PubMed  Google Scholar 

  23. Robson JC, Kiran A, Maskell J, Hutchings A, Arden N, Dasgupta B, et al. The relative risk of aortic aneurysm in patients with giant cell arteritis compared with the general population of the UK. Ann Rheum Dis. 2015;74(1):129–35.

    Article  PubMed  Google Scholar 

  24. Mackie SL, Hensor EM, Morgan AW, Pease CT. Should I send my patient with previous giant cell arteritis for imaging of the thoracic aorta? A systematic literature review and meta-analysis. Ann Rheum Dis. 2014;73(1):143–8.

    Article  PubMed  Google Scholar 

  25. Mwipatayi BP, Jeffery PC, Beningfield SJ, Matley PJ, Naidoo NG, Kalla AA, et al. Takayasu arteritis: clinical features and management: report of 272 cases. ANZ J Surg. 2005;75(3):110–7.

    Article  PubMed  Google Scholar 

  26. Salvarani C, Della Bella C, Cimino L, Macchioni P, Formisano D, Bajocchi G, et al. Risk factors for severe cranial ischaemic events in an Italian population-based cohort of patients with giant cell arteritis. Rheumatology (Oxford). 2009;48(3):250–3.

    Article  CAS  Google Scholar 

  27. Labarca C, Koster MJ, Crowson CS, Makol A, Ytterberg SR, Matteson EL, et al. Predictors of relapse and treatment outcomes in biopsy-proven giant cell arteritis: a retrospective cohort study. Rheumatology (Oxford). 2016;55(2):347–56.

    Article  Google Scholar 

  28. da Silva TF, Levy-Neto M, Bonfá E, Pereira RM. High prevalence of metabolic syndrome in Takayasu arteritis: increased cardiovascular risk and lower adiponectin serum levels. J Rheumatol. 2013;40(11):1897–904.

    Article  PubMed  CAS  Google Scholar 

  29. Durand M, Thomas SL. Incidence of infections in patients with giant cell arteritis: a cohort study. Arthritis Care Res (Hoboken). 2012;64(4):581–8.

    Article  Google Scholar 

  30. Udayakumar PD, Chandran AK, Crowson CS, Warrington KJ, Matteson EL. Hospitalized infections in giant cell arteritis: a population-based retrospective cohort study. J Rheumatol. 2014;41(12):2447–51.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Schmidt J, Smail A, Roche B, Gay P, Salle V, Pellet H, et al. Incidence of severe infections and infection-related mortality during the course of Giant cell arteritis: a multicenter, prospective. Double-Cohort Study Arthritis Rheumatol. 2016;68(6):1477–82.

    Article  CAS  PubMed  Google Scholar 

  32. Gaffo AL. Thrombosis in vasculitis. Best Pract Res Clin Rheumatol. 2013;27(1):57–67.

    Article  CAS  PubMed  Google Scholar 

  33. Emmi G, Silvestri E, Squatrito D, Amedei A, Niccolai E, D’Elios MM, et al. Thrombosis in vasculitis: from pathogenesis to treatment. Thromb J. 2015;13:15.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  34. Aviña-Zubieta JA, Bhole VM, Amiri N, Sayre EC, Choi HK. The risk of deep venous thrombosis and pulmonary embolism in giant cell arteritis: a general population-based study. Ann Rheum Dis. 2016;75(1):148–54.

    Article  PubMed  Google Scholar 

  35. Unizony S, Menendez ME, Rastalsky N, Stone JH. Inpatient complications in patients with giant cell arteritis: decreased mortality and increased risk of thromboembolism, delirium and adrenal insufficiency. Rheumatology (Oxford). 2015;54(8):1360–8.

    Article  Google Scholar 

  36. Kermani TA, Schäfer VS, Crowson CS, Hunder GG, Ytterberg SR, Matteson EL, et al. Cancer preceding giant cell arteritis: a case-control study. Arthritis Rheum. 2010;62(6):1763–9.

    Article  PubMed  PubMed Central  Google Scholar 

  37. Kermani TA, Schäfer VS, Crowson CS, Hunder GG, Gabriel SE, Ytterberg SR, et al. Malignancy risk in patients with giant cell arteritis: a population-based cohort study. Arthritis Care Res (Hoboken). 2010;62(2):149–54.

    PubMed Central  Google Scholar 

  38. Gonzalez-Gay MA, Lopez-Diaz MJ, Martinez-Lado L, Peña-Sagredo JL, Lopez-Agreda H, Miranda-Filloy JA, et al. Cancer in biopsy-proven giant cell arteritis. A population-based study. Semin Arthritis Rheum. 2007;37(3):156–63.

    Article  PubMed  Google Scholar 

  39. Ungprasert P, Sanguankeo A, Upala S, Knight EL. Risk of malignancy in patients with giant cell arteritis and polymyalgia rheumatica: a systematic review and meta-analysis. Semin Arthritis Rheum. 2014;44(3):366–70.

    Article  PubMed  Google Scholar 

  40. Liozon E, Loustaud V, Fauchais AL, Soria P, Ly K, Ouattara B, et al. Concurrent temporal (giant cell) arteritis and malignancy: report of 20 patients with review of the literature. J Rheumatol. 2006;33(8):1606–14.

    PubMed  Google Scholar 

  41. Park JK, Choi IA, Lee EY, Song YW, Lee EB. Incidence of malignancy in Takayasu arteritis in Korea. Rheumatol Int. 2014;34(4):517–21.

    Article  PubMed  Google Scholar 

  42. Cassling RS, Lortz JB, Olson DR, Hubbard TF, McManus BM. Fatal vasculitis (periarteritis nodosa) of the coronary arteries: angiographic ambiguities and absence of aneurysms at autopsy. J Am Coll Cardiol. 1985;6(3):707–14.

    Article  CAS  PubMed  Google Scholar 

  43. Kastner D, Gaffney M, Tak T. Polyarteritis nodosa and myocardial infarction. Can J Cardiol. 2000;16:515–8.

    CAS  PubMed  Google Scholar 

  44. Zoller B, Li X, Sundquist J, Sundquist K. Risk of pulmonary embolism in patients with autoimmune disorders: a nationwide follow-up study from Sweden. Lancet. 2012;379:244–9.

    Article  PubMed  Google Scholar 

  45. Zöller B, Li X, Sundquist J, Sundquist K. Risk of subsequent ischemic and hemorrhagic stroke in patients hospitalized for immune-mediated diseases: a nationwide follow-up study from Sweden. BMC Neurol. 2012;12:41.

    Article  PubMed  PubMed Central  Google Scholar 

  46. Zöller B, Li X, Sundquist J, Sundquist K. Risk of subsequent coronary heart disease in patients hospitalized for immune-mediated diseases: a nationwide follow-up study from Sweden. PLoS One. 2012;7:e33442.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  47. Allenbach Y, Seror R, Pagnoux C, Teixeira L, Guilpain P. Guillevin L; French Vasculitis study group. High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener’s granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients. Ann Rheum Dis. 2009;68(4):564–7.

    Article  CAS  PubMed  Google Scholar 

  48. Hasler P, Kistler H, Gerber H. Vasculitides in hairy cell leukemia. Semin Arthritis Rheum. 1995;25(2):134–42.

    Article  CAS  PubMed  Google Scholar 

  49. Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, et al. French Vasculitis study group. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis study group database. Arthritis Rheum. 2010;62(2):616–26.

    Article  PubMed  Google Scholar 

  50. Guillevin L, Mahr A, Callard P, Godmer P, Pagnoux C, Leray E, et al; French Vasculitis Study Group. Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients. Medicine (Baltimore) 2005;84(5):313–322.

    Google Scholar 

  51. Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Puéchal X, et al. French Vasculitis study group. Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: a prospective randomized study of one hundred twenty-four patients. Arthritis Rheum. 2010;62(4):1186–97.

    Article  PubMed  Google Scholar 

  52. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488–98.

    Article  CAS  PubMed  Google Scholar 

  53. Flossmann O, Berden A, de Groot K, Hagen C, Harper L, Heijl C, et al. European Vasculitis Study Group. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70(3):488–94.

    Article  PubMed  Google Scholar 

  54. Morgan MD, Turnbull J, Selamet U, Kaur-Hayer M, Nightingale P, Ferro CJ, et al. Increased incidence of cardiovascular events in patients with antineutrophil cytoplasmic antibody-associated vasculitides: a matched-pair cohort study. Arthritis Rheum. 2009;60(11):3493–500.

    Article  PubMed  Google Scholar 

  55. Tervaert JW. Translational mini-review series on immunology of vascular disease: accelerated atherosclerosis in vasculitis. Clin Exp Immunol. 2009;156(3):377–85.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  56. Faurschou M, Mellemkjaer L, Sorensen IJ, Svalgaard Thomsen B, Dreyer L, et al. Increased morbidity from ischemic heart disease in patients with Wegener’s granulomatosis. Arthritis Rheum. 2009;60(4):1187–92.

    Article  PubMed  Google Scholar 

  57. Suppiah R, Judge A, Batra R, Flossmann O, Harper L, Höglund P, et al. A model to predict ardiovascular events in patients with newly diagnosed Wegener’s granulomatosis and microscopic polyangiitis. Arthritis Care Res (Hoboken). 2011;63(4):588–96.

    Article  Google Scholar 

  58. de Leeuw K, Sanders JS, Stegeman C, Smit A, Kallenberg CG, Bijl M. Accelerated atherosclerosis in patients with Wegener’s granulomatosis. Ann Rheum Dis. 2005;64(5):753–9.

    Article  PubMed  Google Scholar 

  59. Chironi G, Pagnoux C, Simon A, Pasquinelli-Balice M, Del-Pino M, Gariepy J, et al. Increased prevalence of subclinical atherosclerosis in patients with small-vessel vasculitis. Heart. 2007;93(1):96–9.

    Article  CAS  PubMed  Google Scholar 

  60. Filer AD, Gardner-Medwin JM, Thambyrajah J, Raza K, Carruthers DM, Stevens RJ, et al. Diffuse endothelial dysfunction is common to ANCA associated systemic vasculitis and polyarteritis nodosa. Ann Rheum Dis. 2003;62(2):162–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  61. Hajj-Ali RA, Major J, Langford C, Hoffman GS, Clark T, Zhang L, et al. The interface of inflammation and subclinical atherosclerosis in granulomatosis with polyangiitis (Wegener’s): a preliminary study. Transl Res. 2015;166(4):366–74.

    Article  PubMed  PubMed Central  Google Scholar 

  62. Faurschou M, Obel N, Baslund B. High risk of pulmonary embolism and deep venous thrombosis but not of stroke in granulomatosis with polyangiitis (Wegener’s). Arthritis Care Res (Hoboken). 2014;66(12):1910–4.

    Article  Google Scholar 

  63. Petermann Smits DR, Wilde B, Kianersi Adegani M, de Jongh H, van Paassen P, Cohen Tervaert JW. Metabolic syndrome in ANCA-associated vasculitis. Rheumatology (Oxford). 2013;52(1):197–203.

    Article  Google Scholar 

  64. Merkel PA, Lo GH, Holbrook JT, Tibbs AK, Allen NB, Davis JC Jr, et al. Wegener’s Granulomatosis Etanercept Trial Research Group. Brief communication: high incidence of venous thrombotic events among patients with Wegener granulomatosis: the Wegener’s Clinical Occurrence of Thrombosis (WeCLOT) Study. Ann Intern Med. 2005;142(8):620–6.

    Article  PubMed  Google Scholar 

  65. Weidner S, Hafezi-Rachti S, Rupprecht HD. Thromboembolic events as a complication of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2006;55(1):146–9.

    Article  PubMed  Google Scholar 

  66. Stassen PM, Derks RP, Kallenberg CG, Stegeman CA. Venous thromboembolism in ANCA-associated vasculitis--incidence and risk factors. Rheumatology (Oxford). 2008;47(4):530–4.

    Article  CAS  Google Scholar 

  67. Whyte AF, Smith WB, Sinkar SN, Kette FE, Hissaria P. Clinical and laboratory characteristics of 19 patients with Churg-Strauss syndrome from a single South Australian centre. Intern Med J. 2013;43(7):784–90.

    Article  CAS  PubMed  Google Scholar 

  68. Ames PR, Margaglione M, Mackie S, Alves JD. Eosinophilia and thrombophilia in Churg Strauss syndrome: a clinical and pathogenetic overview. Clin Appl Thromb Hemost. 2010;16(6):628–36.

    Article  CAS  PubMed  Google Scholar 

  69. Little MA, Nightingale P, Verburgh CA, Hauser T, De Groot K, Savage C, et al. European Vasculitis Study (EUVAS) Group. Early mortality in systemic vasculitis: relative contribution of adverse events and active vasculitis. Ann Rheum Dis. 2010;69(6):1036–43.

    Article  PubMed  Google Scholar 

  70. Wall N, Harper L. Complications of long-term therapy for ANCA-associated systemic vasculitis. Nat Rev Nephrol. 2012;8(9):523–32.

    Article  CAS  PubMed  Google Scholar 

  71. Lai QY, Ma TT, Li ZY, Chang DY, Zhao MH, Chen M. Predictors for mortality in patients with antineutrophil cytoplasmic autoantibody-associated vasculitis: a study of 398 Chinese patients. J Rheumatol. 2014;41(9):1849–55.

    Article  PubMed  Google Scholar 

  72. Holle JU, Gross WL, Latza U, Nölle B, Ambrosch P, Heller M, et al. Improved outcome in 445 patients with Wegener’s granulomatosis in a German vasculitis center over four decades. Arthritis Rheum. 2011;63(1):257–66.

    Article  PubMed  Google Scholar 

  73. Cartin-Ceba R, Golbin JM, Keogh KA, Peikert T, Sánchez-Menéndez M, Ytterberg SR, et al. Rituximab for remission induction and maintenance in refractory granulomatosis with polyangiitis (Wegener’s): ten-year experience at a single center. Arthritis Rheum. 2012;64(11):3770–8.

    Article  CAS  PubMed  Google Scholar 

  74. Charlier C, Henegar C, Launay O, Pagnoux C, Berezné A, Bienvenu B, et al. Risk factors for major infections in Wegener granulomatosis: analysis of 113 patients. Ann Rheum Dis. 2009;68(5):658–63.

    Article  CAS  PubMed  Google Scholar 

  75. Aasarød K, Iversen BM, Hammerstrøm J, Bostad L, Vatten L, Jørstad S. Wegener’s granulomatosis: clinical course in 108 patients with renal involvement. Nephrol Dial Transplant. 2000;15(5):611–8.

    Article  PubMed  Google Scholar 

  76. Bligny D, Mahr A, Toumelin PL, Mouthon L, Guillevin L. Predicting mortality in systemic Wegener’s granulomatosis: a survival analysis based on 93 patients. Arthritis Rheum. 2004;51(1):83–91.

    Article  PubMed  Google Scholar 

  77. McGregor JG, Negrete-Lopez R, Poulton CJ, Kidd JM, Katsanos SL, Goetz L, et al. Adverse events and infectious burden, microbes and temporal outline from immunosuppressive therapy in antineutrophil cytoplasmic antibody-associated vasculitis with native renal function. Nephrol Dial Transplant. 2015;30 Suppl 1:Si171–81.

    Article  Google Scholar 

  78. Kitagawa K, Furuichi K, Sagara A, Shinozaki Y, Kitajima S, Toyama T, et al. Kanazawa Study Group for Renal Diseases and Hypertension. Risk factors associated with relapse or infectious complications in Japanese patients with microscopic polyangiitis. Clin Exp Nephrol. 2016;20(5):703–11.

    Article  PubMed  Google Scholar 

  79. Harper L, Savage CO. ANCA-associated renal vasculitis at the end of the twentieth century-a disease of older patients. Rheumatology. 2005;44:495–501.

    Article  CAS  PubMed  Google Scholar 

  80. Booth AD, Almond MK, Burns A, Ellis P, Gaskin G, Neild GH, et al. Pan-Thames Renal Research Group. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis. 2003;41(4):776–84.

    Article  PubMed  Google Scholar 

  81. McGregor JG, Hogan SL, Hu Y, Jennette CE, Falk RJ, Nachman PH. Glucocorticoids and relapse and infection rates in anti-neutrophil cytoplasmic antibody disease. Clin J Am Soc Nephrol. 2012;7(2):240–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  82. Turnbull J, Harper L. Adverse effects of therapy for ANCA-associated vasculitis. Best Pract Res Clin Rheumatol. 2009;23(3):391–401.

    Article  PubMed  Google Scholar 

  83. Guillevin L, Cordier JF, Lhote F, Cohen P, Jarrousse B, Royer I, et al. A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener’s granulomatosis. Arthritis Rheum. 1997;40(12):2187–98.

    Article  CAS  PubMed  Google Scholar 

  84. Nossent JC. Rituximab or cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363(21):2072–4. author reply 2073-4

    Article  CAS  PubMed  Google Scholar 

  85. Besada E, Koldingsnes W, Nossent JC. Long-term efficacy and safety of pre-emptive maintenance therapy with rituximab in granulomatosis with polyangiitis: results from a single centre. Rheumatology. 2013;52(11):2041–7.

    Article  CAS  PubMed  Google Scholar 

  86. Alberici F, Smith RM, Jones RB, Roberts DM, Willcocks LC, Chaudhry A, et al. Long-term follow-up of patients who received repeat-dose rituximab as maintenance therapy for ANCA-associated vasculitis. Rheumatology (Oxford). 2015;54(7):1153–60.

    Article  Google Scholar 

  87. Marco H, Smith RM, Jones RB, Guerry MJ, Catapano F, Burns S, et al. The effect of rituximab therapy on immunoglobulin levels in patients with multisystem autoimmune disease. BMC Musculoskelet Disord. 2014;15:178.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  88. Venhoff N, Effelsberg NM, Salzer U, Warnatz K, Peter HH, Lebrecht D, et al. Impact of rituximab on immunoglobulin concentrations and B cell numbers after cyclophosphamide treatment in patients with ANCA-associated vasculitides. PLoS One. 2012;7(5):e37626.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  89. Besada E, Koldingsnes W, Nossent JC. Serum immunoglobulin levels and risk factors for hypogammaglobulinemia during long-term maintenance therapy with rituximab in patients with granulomatosis with polyangiitis. Rheumatology. 2014;53(10):1818–24.

    Article  CAS  PubMed  Google Scholar 

  90. Knight A, Sundström Y, Börjesson O, Bruchfeld A, Malmström V, Gunnarsson I. Late-onset neutropenia after rituximab in ANCA-associated vasculitis. Scand J Rheumatol. 2016;7:1–4.

    Google Scholar 

  91. Gayraud M, Guillevin L, Cohen P, Lhote F, Cacoub P, Deblois P, et al. Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides. Br J Rheumatol. 1997;36(12):1290–7.

    Article  CAS  PubMed  Google Scholar 

  92. Haubitz M, Schellong S, Göbel U, Schurek HJ, Schaumann D, Koch KM, et al. Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmic antibody-associated vasculitis and renal involvement: a prospective, randomized study. Arthritis Rheum. 1998;41(10):1835–44.

    Article  CAS  PubMed  Google Scholar 

  93. Adu D, Pall A, Luqmani RA, Richards NT, Howie AJ, Emery P, et al. Controlled trial of pulse versus continuous prednisolone and cyclophosphamide in the treatment of systemic vasculitis. QJM. 1997;90(6):401–9.

    Article  CAS  PubMed  Google Scholar 

  94. Edgar JD, Rooney DP, McNamee P, McNeill TA. An association between ANCA positive renal disease and malignancy. Clin Nephrol. 1993;40(1):22–5.

    CAS  PubMed  Google Scholar 

  95. Westman KW, Bygren PG, Olsson H, Ranstam J, Wieslander J. Relapse rate, renal survival, and cancer morbidity in patients with Wegener’s granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol. 1998;9(5):842–52.

    CAS  PubMed  Google Scholar 

  96. Knight A, Askling J, Ekbom A. Cancer incidence in a population-based cohort of patients with Wegener’s granulomatosis. Int J Cancer. 2002;100(1):82–5.

    Article  CAS  PubMed  Google Scholar 

  97. Pankhurst T, Savage CO, Gordon C, Harper L. Malignancy is increased in ANCA-associated vasculitis. Rheumatology (Oxford). 2004;43(12):1532–5.

    Article  CAS  Google Scholar 

  98. Tatsis E, Reinhold-Keller E, Steindorf K, Feller AC, Gross WL. Wegener’s granulomatosis associated with renal cell carcinoma. Arthritis Rheum. 1999;42(4):751–6.

    Article  CAS  PubMed  Google Scholar 

  99. Rahmattulla C, Berden AE, Wakker SC, Reinders ME, Hagen EC, Wolterbeek R, et al. Incidence of malignancies in patients with antineutrophil cytoplasmic antibody-associated vasculitis diagnosed between 1991 and 2013. Arthritis Rheumatol. 2015;67(12):3270–8.

    Article  CAS  PubMed  Google Scholar 

  100. Faurschou M, Mellemkjaer L, Voss A, Keller KK, Hansen IT, Baslund B. Prolonged risk of specific malignancies following cyclophosphamide therapy among patients with granulomatosis with polyangiitis. Rheumatology (Oxford). 2015;54(8):1345–50.

    Article  Google Scholar 

  101. Shang W, Ning Y, Xu X, Li M, Guo S, Han M, et al. Incidence of cancer in ANCA-associated vasculitis: a meta-analysis of observational studies. PLoS One. 2015;10(5):e0126016.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  102. Charlson ME, Pompei P, Ales KL, MacKenzie CR. A new method of classifying prognostic comorbidity in longitudinal studies: development and validation. J Chronic Dis. 1987;40(5):373–83.

    Article  CAS  PubMed  Google Scholar 

  103. Charlson M, Szatrowski TP, Peterson J, Gold J. Validation of a combined comorbidity index. J Clin Epidemiol. 1994;47(11):1245–51.

    Article  CAS  PubMed  Google Scholar 

  104. Deyo RA, Cherkin DC, Ciol MA. Adapting a clinical comorbidity index for use with ICD-9-CM administrative databases. J Clin Epidemiol. 1992;45(6):613–9.

    Article  CAS  PubMed  Google Scholar 

  105. Ofer-Shiber S, Molad Y. Association of the Charlson comorbidity index with renal outcome and all-cause mortality in antineutrophil cytoplasmatic antibody-associated vasculitis. Medicine (Baltimore). 2014;93(25):e152.

    Article  Google Scholar 

  106. Faurschou M, Ahlström MG, Lindhardsen J, Baslund B, Obel N. Impact of pre-existing co-morbidities on mortality in granulomatosis with polyangiitis: a cohort study. Rheumatology (Oxford). 2016;55(4):649–53.

    Article  Google Scholar 

  107. Hatemi G, Yazici Y, Yazici H. Behçet’s syndrome. Rheum Dis Clin N Am. 2013;39(2):245–61.

    Article  Google Scholar 

  108. Sazzini M, Garagnani P, Sarno S, De Fanti S, Lazzano T, Yang Yao D, et al. Tracing Behçet’s disease origins along the silk road: an anthropological evolutionary genetics perspective. Clin Exp Rheumatol. 2015;33(6 Suppl 94):S60–6.

    PubMed  Google Scholar 

  109. Gul A, Ohno S. HLA-B*51 and Behçet disease. Ocul Immunol Inflamm. 2012;20(1):37–43.

    Article  CAS  PubMed  Google Scholar 

  110. Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A, et al. EULAR Expert Committee. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008;67(12):1656–62.

    Article  CAS  PubMed  Google Scholar 

  111. Demirelli S, Degirmenci H, Inci S, Arisoy A. Cardiac manifestations in Behcet’s disease. Intractable Rare Dis Res. 2015;4(2):70–5.

    Article  PubMed  PubMed Central  Google Scholar 

  112. Geri G, Wechsler B, Thi Huon du L, Isnard R, Piette JC, Amoura Z, et al. Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature. Medicine (Baltimore) 2012;91(1):25–34.

    Google Scholar 

  113. Fei Y, Li X, Lin S, Song X, Wu Q, Zhu Y, et al. Major vascular involvement in Behçet’s disease: a retrospective study of 796 patients. Clin Rheumatol. 2013;32(6):845–52.

    Article  PubMed  Google Scholar 

  114. Seyahi E, Yazici H. Behçet’s syndrome: pulmonary vascular disease. Curr Opin Rheumatol. 2015;27(1):18–23.

    Article  PubMed  Google Scholar 

  115. Celik S, Yazici Y, Sut N, Yazici H. Pulmonary artery aneurysms in Behçet’s syndrome: a review of the literature with emphasis on geographical differences. Clin Exp Rheumatol. 2015;33(6 Suppl 94):S54–9.

    PubMed  Google Scholar 

  116. Yazici H, Esen F. Mortality in Behçet’s syndrome. Clin Exp Rheumatol. 2008;26(5 Suppl 51):S138–40.

    CAS  PubMed  Google Scholar 

  117. Saadoun D, Wechsler B, Desseaux K, Le Thi HD, Amoura Z, Resche-Rigon M, et al. Mortality in Behçet’s disease. Arthritis Rheum. 2010;62(9):2806–12.

    Article  CAS  PubMed  Google Scholar 

  118. Yang SS, Park KM, Park YJ, Kim YW, Do YS, Park HS, et al. Peripheral arterial involvement in Behcet’s disease: an analysis of the results from a Korean referral center. Rheumatol Int. 2013;33(8):2101–8.

    Article  PubMed  Google Scholar 

  119. Seyahi E, Ugurlu S, Cumali R, Balci H, Ozdemir O, Melikoglu M, et al. Atherosclerosis in Behçet’s syndrome. Semin Arthritis Rheum. 2008;38(1):1–12.

    Article  CAS  PubMed  Google Scholar 

  120. Marzban M, Mandegar MH, Karimi A, Abbasi K, Movahedi N, Navabi MA, et al. Cardiac and great vessel involvement in “Behcet’s disease”. J Card Surg. 2008;23(6):765–8.

    Article  PubMed  Google Scholar 

  121. Ugurlu S, Seyahi E, Yazici H. Prevalence of angina, myocardial infarction and intermittent claudication assessed by Rose Questionnaire among patients with Behcet’s syndrome. Rheumatology (Oxford). 2008;47(4):472–5.

    Article  CAS  Google Scholar 

  122. Aslam F, Bandeali SJ, Crowson C, Alam M. Cardiac function and diastolic dysfunction in Behcet’s disease: a systematic review and meta-analysis. Int J Rheumatol. 2016;2016:9837184.

    Article  PubMed  PubMed Central  Google Scholar 

  123. Tascilar K, Melikoglu M, Ugurlu S, Sut N, Caglar E, Yazici H. Vascular involvement in Behçet’s syndrome: a retrospective analysis of associations and the time course. Rheumatology (Oxford). 2014;53(11):2018–22.

    Article  CAS  Google Scholar 

  124. Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A, Akhlaghi M, et al. Behcet’s disease: from East to West. Clin Rheumatol. 2010;29(8):823–33.

    Article  PubMed  Google Scholar 

  125. Koç Y, Güllü I, Akpek G, Akpolat T, Kansu E, Kiraz S, et al. Vascular involvement in Behçet’s disease. J Rheumatol. 1992;19(3):402–10.

    PubMed  Google Scholar 

  126. Gürler A, Boyvat A, Türsen U. Clinical manifestations of Behçet’s disease: an analysis of 2147 patients. Yonsei Med J. 1997;38(6):423–7.

    Article  PubMed  Google Scholar 

  127. Ames PR, Steuer A, Pap A, Denman AM. Thrombosis in Behçet’s disease: a retrospective survey from a single UK center. Rheumatology (Oxford). 2001;40(6):652–5.

    Article  CAS  Google Scholar 

  128. Sarica-Kucukoglu R, Akdag-Kose A, KayabalI M, Yazganoglu KD, Disci R, Erzengin D, et al. Vascular involvement in Behçet’s disease: a retrospective analysis of 2319 cases. Int J Dermatol. 2006;45(8):919–21.

    Article  PubMed  Google Scholar 

  129. La Regina M, Gasparyan AY, Orlandini F, Prisco D. Behcet’s disease as a model of venous thrombosis. Open Cardiovasc Med J. 2010;4:71–7.

    PubMed  PubMed Central  Google Scholar 

  130. Seyahi E, Caglar E, Ugurlu S, Kantarci F, Hamuryudan V, Sonsuz A, et al. An outcome survey of 43 patients with Budd-Chiari syndrome due to Behçet’s syndrome followed up at a single, dedicated center. Semin Arthritis Rheum. 2015;44(5):602–9.

    Article  PubMed  Google Scholar 

  131. Desbois AC, Rautou PE, Biard L, Belmatoug N, Wechsler B, Resche-Rigon M, et al. Behcet’s disease in Budd-Chiari syndrome. Orphanet J Rare Dis. 2014;9:104.

    Article  PubMed  PubMed Central  Google Scholar 

  132. Fernández-Bello I, López-Longo FJ, Arias-Salgado EG, Jiménez-Yuste V, Butta NV. Behçet’s disease: new insight into the relationship between procoagulant state, endothelial activation/damage and disease activity. Orphanet J Rare Dis. 2013;8:81.

    Article  PubMed  PubMed Central  Google Scholar 

  133. Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi HD, Sbai A, et al. Cerebral venous thrombosis in Behçet’s disease. Arthritis Rheum. 2009;61(4):518–26.

    Article  CAS  PubMed  Google Scholar 

  134. Aguiar de Sousa D, Mestre T, Ferro JM. Cerebral venous thrombosis in Behçet’s disease: a systematic review. J Neurol. 2011;258(5):719–27.

    Article  CAS  PubMed  Google Scholar 

  135. Alexoudi I, Kapsimali V, Vaiopoulos A, Kanakis M, Vaiopoulos G. Evaluation of current therapeutic strategies in Behçet’s disease. Clin Rheumatol. 2011;30(2):157–63.

    Article  PubMed  Google Scholar 

  136. Seyahi E, Melikoglu M, Akman C, Hamuryudan V, Ozer H, Hatemi G, et al. Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients. Medicine (Baltimore). 2012;91(1):35–48.

    Article  Google Scholar 

  137. Mogulkoc N, Burgess MI, Bishop PW. Intracardiac thrombus in Behçet’s disease: a systematic review. Chest. 2000;118(2):479–87.

    Article  CAS  PubMed  Google Scholar 

  138. Vallet H, Riviere S, Sanna A, Deroux A, Moulis G, Addimanda O. French Behçet Network. Efficacy of anti-TNF alpha in severe and/or refractory Behçet’s disease: multicenter study of 124 patients. J Autoimmun. 2015;62:67–74.

    Article  CAS  PubMed  Google Scholar 

  139. Wang LH, Wang WM, Hsu SM, Lin SH, Shieh CC. Risk of overall and site-specific cancers in Behçet disease: a nationwide population-based study in Taiwan. J Rheumatol. 2015;42(5):879–84.

    Article  PubMed  Google Scholar 

  140. Ahn JK, Oh JM, Lee J, Koh EM, Cha HS. Behcet’s disease associated with malignancy in Korea: a single center experience. Rheumatol Int. 2010;30(6):831–5.

    Article  PubMed  Google Scholar 

  141. Lin Y, Li G, Zheng W, Tian X, Zhang F. Behcet’s disease associated with malignancy: a report of 41 Chinese cases. Int J Rheum Dis. 2014;17(4):459–65.

    Article  PubMed  Google Scholar 

  142. Cheon JH, Celik AF, Kim WH. Behcet’s disease: gastrointestinal involvement. In: Yazici Y, Yazici H, editors. Behcet’s syndrome. New York: Springer; 2010. p. 165–88.

    Chapter  Google Scholar 

  143. Yurdakul S, Tüzüner N, Yurdakul I, Hamuryudan V, Yazici H. Gastrointestinal involvement in Behçet’s syndrome: a controlled study. Ann Rheum Dis. 1996;55(3):208–10.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  144. Esatoglu SN, Hatemi G, Salihoglu A, Hatemi I, Soysal T, Celik AF. A reappraisal of the association between Behçet’s disease, myelodysplastic syndrome and the presence of trisomy 8: a systematic literature review. Clin Exp Rheumatol. 2015;33(6 Suppl 94):S145–51.

    PubMed  Google Scholar 

  145. Tada Y, Koarada S, Haruta Y, Mitamura M, Ohta A, Nagasawa K. The association of Behçet’s disease with myelodysplastic syndrome in Japan: a review of the literature. Clin Exp Rheumatol. 2006;24(5 Suppl 42):S115–9.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Rheumatology Unit, Rabin Medical Center – Beilinson Hospital, Petach Tikva and Sackler Faculty of Medicine, Tel Aviv, Israel

    Yair Molad

Authors
  1. Yair Molad
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Yair Molad .

Editor information

Editors and Affiliations

  1. Consultant Rheumatologist, Department of Rheumatology, Darent Valley Hospital, Dartford, Kent, United Kingdom

    Yasser El Miedany

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing AG

About this chapter

Cite this chapter

Molad, Y. (2017). Vasculitis. In: El Miedany, Y. (eds) Comorbidity in Rheumatic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-59963-2_12

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-59963-2_12

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-59962-5

  • Online ISBN: 978-3-319-59963-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation