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Systemic and central nervous system vasculitides

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Opinion statement

The vasculitides are a collection of diseases sharing the central feature of tissue injury due to inflammation of blood vessels. Although some occur frequently, many are encountered uncommonly or rarely. Nonetheless, because peripheral or central nervous system abnormalities may be a prominent part of these disorders, neurologists should know or be able to refer to studies on the classification, pathophysiology, treatments, and long-term complications of vasculitis. Two central issues that confront us now are 1) improving the accuracy of diagnosis, including identifying any underlying infectious causes, and 2) limiting the long-term damage from both the disease and its therapies.

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References and Recommended Reading

  1. Moore PM, Richardson B: Neurology of the vasculitides and connective tissue diseases. J Neurol Neurosurg Psychiatry 1998, 65:10–22. Review of the vasculitides and their neurologic complications.

    PubMed  CAS  Google Scholar 

  2. Scott DG, Watts RA: Classification and epidemiology of systemic vasculitis. Br J Rheumatol 1994, 33:897–899.

    Article  PubMed  CAS  Google Scholar 

  3. Juvonen T, Juvonen J, Savolainen MJ: Is vasculitis a significant component of atherosclerosis? Curr Opin Rheumatol 1999, 11:3–10.

    Article  PubMed  CAS  Google Scholar 

  4. Cohen T, Popa ER, Bos NA: The role of superantigens in vasculitis. Curr Opin Rheumatol 1999, 11:24–33.

    Article  Google Scholar 

  5. Lynch JP, Hoffman GS: Wegener’s granulomatosis: controversies and current concepts. Compr Ther 1998, 24:421–440. Thoughtful discussion of the problems in adapting an effective therapy to individual aspects of a disease.

    PubMed  Google Scholar 

  6. Gross WL: New concepts in treatment protocols for severe systemic vasculitis. Curr Opin Rheumatol 1999, 11:41–46.

    Article  PubMed  CAS  Google Scholar 

  7. Sneller MC: Evaluation, treatment, and prophylaxis of infections complicating systemic vasculitis. Curr Opin Rheumatol 1998, 10:38–44. Excellent review for anyone using immune system medications, which compromise the body’s ability to deal with both bacterial and opportunistic infections.

    Article  PubMed  CAS  Google Scholar 

  8. Exley AR, Bacon PA, Luqmani RA, et al.: Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum 1997, 40:371–380.

    Article  PubMed  CAS  Google Scholar 

  9. Hoffman GS, Drucker Y, Cotch MF, et al.: Wegener’s granulomatosis: patient-reported effects of disease on health, function, and income. Arthritis Rheum 1998, 41:2257–2262.

    Article  PubMed  CAS  Google Scholar 

  10. Abu-Shakra M, Smythe H, Lewtas J, et al.: Outcome of polyarteritis nodosa and Churg-Strauss syndrome. An analysis of twenty-five patients. Arthritis Rheum 1994, 37:1798–1803.

    Article  PubMed  CAS  Google Scholar 

  11. Gordon M, Luqmani RA, Adu D, et al.: Relapses in patients with a systemic vasculitis. Q J Med 1993, 86:779–789.

    PubMed  CAS  Google Scholar 

  12. Hoffman GS, Kerr GS, Leavitt RY, et al.: Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992, 116:488–498.

    PubMed  CAS  Google Scholar 

  13. Brack A, Martinez-Taboada V, Stanson A, et al.: Disease pattern in cranial and large-vessel giant cell arteritis. Arthritis Rheum 1999, 42:311–317.

    Article  PubMed  CAS  Google Scholar 

  14. Adu D, Pall A, Luqmani RA, et al.: Controlled trial of pulse versus continuous prednisolone and cyclophosphamide in the treatment of systemic vasculitis. QJM 1997, 90:401–409.

    Article  PubMed  CAS  Google Scholar 

  15. Haubitz M, Schellong S, Gobel U, 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, 41:1835–1844.

    Article  PubMed  CAS  Google Scholar 

  16. Stegeman CA, Cohen Tervaert JW, de Jong PE, Kallenberg CG: Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med 1996, 335:16–20.

    Article  PubMed  CAS  Google Scholar 

  17. Fortin PR, Larson MG, Watters AK, et al.: Prognostic factors in systemic necrotizing vasculitis of the polyarteritis nodosa group—a review of 45 cases. J Rheumatol 1995, 22:78–84.

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  19. Sehgal M, Swanson JW, DeRemee RA, Colby TV: Neurologic manifestations of Churg-Strauss syndrome. Mayo Clin Proc 1995, 70:337–341.

    PubMed  CAS  Google Scholar 

  20. Guillevin L, Cohen P, Gayraud M, et al.: Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine 1999, 78:26–37.

    Article  PubMed  CAS  Google Scholar 

  21. Gayraud M, Guillevin L, Cohen 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, 36:1290–1297.

    Article  PubMed  CAS  Google Scholar 

  22. Tatsis E, Schnabel A, Gross WL: Interferon-alpha treatment of four patients with the Churg-Strauss syndrome. Ann Intern Med 1998, 129:370–374.

    PubMed  CAS  Google Scholar 

  23. Cid MC, Font C, Coll-Vinent B, Grau JM: Large vessel vasculitides. Curr Opin Rheumatol 1998, 10:18–28.

    Article  PubMed  CAS  Google Scholar 

  24. Matteson EL, Gold KN, Bloch DA, Hunder GG: Longterm survival of patients with giant cell arteritis in the American College of Rheumatology giant cell arteritis classification criteria cohort. Am J Med 1996, 100:193–196.

    Article  PubMed  CAS  Google Scholar 

  25. Gonzalez-Gay MA, Blanco R, Rodriguez-Valverde V, et al.: Permanent visual loss and cerebrovascular accidents in giant cell arteritis: predictors and response to treatment. Arthritis Rheum 1998, 41:1497–1504.

    Article  PubMed  CAS  Google Scholar 

  26. Rizzi R, Bruno S, Stellacci C, Dammacco R: Takayasu’s arteritis: a cell-mediated large-vessel vasculitis. Int J Clin Lab Res 1999, 29:8–13.

    Article  PubMed  CAS  Google Scholar 

  27. Daina E, Schieppati A, Remuzzi G: Mycophenolate mofetil for the treatment of Takayasu arteritis: report of three cases. Ann Intern Med 1999, 130:422–426.

    PubMed  CAS  Google Scholar 

  28. Tyagi S, Verma PK, Gambhir DS, et al.: Early and longterm results of subclavian angioplasty in aortoarteritis (Takayasu disease): comparison with atherosclerosis. Cardiovasc Intervent Radiol 1998, 21:219–224.

    Article  PubMed  CAS  Google Scholar 

  29. Yazici H, Yurdakul S, Hamuryudan V: Behcet’s syndrome. Curr Opin Rheumatol 1999, 11:53–57.

    Article  PubMed  CAS  Google Scholar 

  30. Hamuryudan V, Ozyazgan Y, Hizli N, et al.: Azathioprine in Behcet’s syndrome: effects on long-term prognosis. Arthritis Rheum 1997, 40:769–774.

    Article  PubMed  CAS  Google Scholar 

  31. Yazici H, Pazarli H, Barnes CG, et al.: A controlled trial of azathioprine in Behcet’s syndrome. N Engl J Med 1997, 322:281–285.

    Article  Google Scholar 

  32. O’Duffy JD, Calamia K, Cohen S, et al.: Interferonalpha treatment of Behcet’s disease. J Rheumatol 1998, 25:1938–1944.

    PubMed  CAS  Google Scholar 

  33. Hamuryudan V, Mat C, Saip S, et al.: Thalidomide in the treatment of the mucocutaneous lesions of the Behcet syndrome. A randomized, double-blind, placebo-controlled trial. Ann Intern Med 1998, 128:443–450.

    PubMed  CAS  Google Scholar 

  34. Hirohata S, Suda H, Hashimoto T: Low-dose weekly methotrexate for progressive neuropsychiatric manifestations in Behcet’s disease. J Neuro Sci 1998, 159:181–185.

    Article  CAS  Google Scholar 

  35. Guillevin L, Lhote F, Gherardi R: The spectrum and treatment of virus-associated vasculitides. Curr Opin Rheumatol 1997, 9:31–36. An important review of the viral diseases that may appear as idiopathic vasculitis.

    Article  PubMed  CAS  Google Scholar 

  36. Misini R, Bellavita P, Fenili D, et al.: Interferon alfa-2a therapy in cryoglobulinemia associated with hepatitis C virus. N Engl J Med 1994, 330:751–756.

    Article  Google Scholar 

  37. Laupland KB, Dele D: Epidemiology, etiology, and management of Kawasaki disease: state of the art. Pediatr Cardiol 1999, 20:177–183.

    Article  PubMed  CAS  Google Scholar 

  38. Burns JC, Capparelli EV, Brown JA, et al.: Intravenous gamma-globulin treatment and retreatment in Kawasaki disease. US/Canadian Kawasaki Syndrome Study Group. Pediatr Infect Dis J 1998, 17:1144–1148.

    Article  PubMed  CAS  Google Scholar 

  39. Merkel PA: Drugs associated with vasculitis. Curr Opin Rheumatol 1998, 10:45–50. An excellent review of the spectrum of causes of secondary vasculitis.

    Article  PubMed  CAS  Google Scholar 

  40. Mehta JL: Salutary effects of aspirin in coronary artery disease are not limited to its platelet inhibitory effects. Clin Cardiol 1999, 21:879–884.

    Article  Google Scholar 

  41. Brooks P, Emery P, Evans JF, et al.: Interpreting the clinical significance of the differential inhibition of cyclooxygenase-1 and cyclooxygenase-2. Rheumatology (Oxford) 1999, 38:779–788.

    Article  CAS  Google Scholar 

  42. Kaplan-Machlis B, Klostermeyer BS: The cyclooxygenase-2 inhibitors: safety and effectiveness. Ann Pharmacother 1999, 33:979–988.

    Article  PubMed  CAS  Google Scholar 

  43. Bahlas S, Ramos-Remus C, Davis P: Clinical outcome of 149 patients with polymyalgia rheumatica and giant cell arteritis. J Rheumatol 1998, 25:99–104.

    PubMed  CAS  Google Scholar 

  44. Bulkley BH, Roberts WC: The heart in systemic lupus erythematosus and the changes induced in it by corticosteroid therapy. A study of 36 necropsy patients. Am J Med 1975, 58:243–264.

    Article  PubMed  CAS  Google Scholar 

  45. Stuck AE, Minder CE, Frey FJ: Risk of infectious complications in patients taking glucocorticosteroids. Rev Infect Dis 1989, 11:954–963.

    PubMed  CAS  Google Scholar 

  46. Conesa D, Rello J, Valles J, et al.: Invasive aspergillosis: a life-threatening complication of short-term steroid treatment. Ann Pharmacother 1995, 29:1235–1237.

    PubMed  CAS  Google Scholar 

  47. Magiakou MA, Chrousos GP: Corticosteroid therapy, nonendocrine disease, and corticosteroid withdrawal. Curr Ther Endocrinol Metab 1997, 6:138–142.

    PubMed  CAS  Google Scholar 

  48. Langford CA, Klippel JH, Balow JE, et al.: Use of cytotoxic agents and cyclosporine in the treatment of autoimmune disease. Part 1: rheumatologic and renal diseases. Ann Intern Med 1998, 128:1021–1028.

    PubMed  CAS  Google Scholar 

  49. Richmond R, McMillan TW, Luqmani RA: Optimisation of cyclophosphamide therapy in systemic vasculitis. Clin Pharmacokinet 1998, 34:79–90.

    Article  PubMed  CAS  Google Scholar 

  50. Guillevin L, Cordier JF, Lhote F, 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, 40:2187–2198.

    Article  PubMed  CAS  Google Scholar 

  51. Bradley JD, Brandt KD, Katz BP: Infectious complications of cyclophosphamide treatment for vasculitis. Arthritis Rheum 1989, 32:45–53.

    Article  PubMed  CAS  Google Scholar 

  52. Haubitz M, Ehlerding C, Kamino K, et al.: Reduced gonadal toxicity after i.v. cyclophosphamide administration in patients with nonmalignant diseases. Clin Nephrol 1998, 49:19–23.

    PubMed  CAS  Google Scholar 

  53. Langford CA: Complications of cyclophosphamide therapy. Eur Arch Otorhinolaryngol 1997, 254:65–72.

    Article  PubMed  CAS  Google Scholar 

  54. Vassallo R, Remstein ED, Parisi JE, et al.: Multiple cerebral infarctions from nonbacterial thrombotic endocarditis mimicking cerebral vasculitis. Mayo Clin Proc 1999, 74:798–802.

    PubMed  CAS  Google Scholar 

  55. Garner BF, Burns P, Bunning RD, Laureno R: Acute blood pressure elevation can mimic arteriographic appearance of cerebral vasculitis (a postpartum case with relative hypertension). J Rheumatol 1990, 17:93–97.

    PubMed  CAS  Google Scholar 

  56. Stone JH, Pomper MG, Hellmann DB: Histoplasmosis mimicking vasculitis of the central nervous system. J Rheumatol 1998, 25:1644–1648.

    PubMed  CAS  Google Scholar 

  57. Fountain NB, Lopes MB: Control of primary angiitis of the CNS associated with cerebral amyloid angiopathy by cyclophosphamide alone. Neurology 1999, 52:660–662.

    PubMed  CAS  Google Scholar 

  58. Hoffman GS: Takayasu arteritis: lessons from the American National Institutes of Health experience. Int J Cardiol 1996, 54(suppl):99–102.

    Article  Google Scholar 

  59. de Groot K, Reinhold-Keller E, Tatsis E, et al.: Therapy for the maintenance of remission in sixty-five patients with generalized Wegener’s granulomatosis. Methotrexate versus trimethoprim/sulfamethoxazole. Arthritis Rheum 1996, 39:2052–2061.

    Article  PubMed  Google Scholar 

  60. Tessler HH, Jennings T: High-dose short-term chlorambucil for intractable sympathetic ophthalmia and Behcet’s disease. Br J Ophthalmol 1990, 74:353–357.

    PubMed  CAS  Google Scholar 

  61. Ishikawa H: Mizoribine and mycophenolate mofetil. Curr Med Chem 1999, 6:575–597.

    PubMed  CAS  Google Scholar 

  62. Waiser J, Budde K, Braasch E, Neumayer HH: Treatment of acute c-ANCA-positive vasculitis with mycophenolate mofetil. Am J Kidney Dis 1999, 34:e9.

    PubMed  CAS  Google Scholar 

  63. Tseng S, Pak G, Washenik K, et al.: Rediscovering thalidomide: a review of its mechanism of action, side effects, and potential uses. J Am Acad Dermatol 1996, 35:969–979.

    Article  PubMed  CAS  Google Scholar 

  64. Gordon AC, Edgar JD, Finch RG: Acute exacerbation of vasculitis during interferon-alpha therapy for hepatitis C-associated cryoglobulinaemia. J Infect 1998, 36:229–230.

    Article  PubMed  CAS  Google Scholar 

  65. Valentine AD, Meyers CA, Kling MA, et al.: Mood and cognitive side effects of interferon-alpha therapy. Semin Oncol 1998, 25(suppl):39–47.

    PubMed  CAS  Google Scholar 

  66. Tambini R, Quattrini A, Fracassetti O, Nemni R: Axonal neuropathy in a patient receiving interferon-alpha therapy for chronic hepatitis C. J Rheumatol 1997, 24:1656–1657.

    PubMed  CAS  Google Scholar 

  67. Rekhter MD, Simari RD, Work CW, et al.: Gene transfer into normal and atherosclerotic human blood vessels. Circ Res 1998, 82:1243–1252.

    PubMed  CAS  Google Scholar 

  68. Stephan D, San H, Yang ZY, et al.: Inhibition of vascular smooth muscle cell proliferation and intimal hyperplasia by gene transfer of beta-interferon. Molec Med 1997, 3:593–599.

    CAS  Google Scholar 

  69. Barron KS, Shulman ST, Rowley A, et al.: Report of the National Institutes of Health Workshop on Kawasaki Disease. J Rheumatol 1999, 26:170–190.

    PubMed  CAS  Google Scholar 

  70. Guillevin L, Fain O, Lhote F, et al.: Lack of superiority of steroids plus plasma exchange to steroids alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome. Arthritis Rheum 1992, 35:208–215.

    Article  PubMed  CAS  Google Scholar 

  71. Bali HK, Jain S, Jain A, Sharma BK: Stent supported angioplasty in Takayasu arteritis. Int J Cardiol 1998, 66(suppl 1):213–217.

    Article  Google Scholar 

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Zivkovic, S., Moore, P.M. Systemic and central nervous system vasculitides. Curr Treat Options Neurol 2, 459–472 (2000). https://doi.org/10.1007/s11940-000-0044-8

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