Treatment of Vasculitides Affecting the Central Nervous System

  • Patricia M. Moore
  • Norbert Gretz
Conference paper


Vasculitis is being increasingly recognized as a cause of neurologic disease. The results of treatment vary from excellent to frustrating depending on the type of vasculitis, the etiology, and the stage of the disease. Some regimens in individual diseases dramatically reduce morbidity and mortality. In other disorders the optimal treatment remains empiric.


Giant Cell Arteritis Thrombotic Thrombocytopenic Purpura Systemic Vasculitis Takayasu Arteritis Polyarteritis Nodosa 
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  1. 1.
    Budzilovich GN, Feigin I, Siegel H (1963) Granulomatous angiitis of the nervous system. Arch Pathol 76:250–256PubMedGoogle Scholar
  2. 2.
    Campion EW (1992) Desperate diseases and plasmapheresis. N Engl J Med 326(21): 1425–1427PubMedCrossRefGoogle Scholar
  3. 3.
    Caselli RJ, Hunder GG, Whisnant JP (1988) Neurologic disease in biopsy-proven giant cell (temporal) arteritis. Neurology 38:352–359PubMedCrossRefGoogle Scholar
  4. 4.
    Clements PJ, Davis J (1986) Cytotoxic drags: their clinical applications to the rheumatic diseases. Semin Arthritis Rheum 15(4):231–254PubMedCrossRefGoogle Scholar
  5. 5.
    Conn DL (1989) Subspeciality clinics: Rheumatology. Update on systemic necrotizing vasculitis. Mayo Clin Proc 64:535–543PubMedCrossRefGoogle Scholar
  6. 6.
    Conn DL, Tompkins RB, Nichols WL (1988) Glucocorticoids in the management of vasculitis - a double edge sword? J Rheumatol 15:1181–1183PubMedGoogle Scholar
  7. 7.
    DeRemee RA (1988) The treatment of Wegener’s granulomatosis with trimethoprim/sulfamethoxazole: illusion or vision? Arthritis Rheum 31(8): 1068–1072PubMedCrossRefGoogle Scholar
  8. 8.
    De Vita SD, Bombardieri S (1991) Cyclophosphamide pulses in the treatment of rheumatic diseases: an update. Clin Exp Rheumatol 9:179–193PubMedGoogle Scholar
  9. 9.
    Dietrich G, Kaveri SV, Kazatchkine MD (1992) Modulation of autoimmunity by intravenous immune globulin through interaction with the function of the immune/ idiotypic network. Clin Immunol Immunopathol 62: S73-S81PubMedCrossRefGoogle Scholar
  10. 10.
    Fauci AS, Wolff SM (1973) Wegener’s granulomatosis: studies in eighteen patients and a review of the literature. Medicine 52:535–561PubMedCrossRefGoogle Scholar
  11. 11.
    Fauci AS, Haynes BF, Costa J, Katz P, Wolff SM (1982) Lymphomatoid granulomatosis. Prospective clinical and therapeutic experience over 10 years. N Engl J Med 306:68–74PubMedCrossRefGoogle Scholar
  12. 12.
    Frank MM, Basta M, Fries LF (1992) The effects of intravenous immune globulin on complement-dependent immune damage of cells and tissues. Clin Immunol Immunopathol 62:982–986CrossRefGoogle Scholar
  13. 13.
    Giulian D, Woodward J, Young DG, Krebs JF, Lachman LB Interleukin-1 injected into mamalian brain stimulates astrogliosis and neovascularization. J Neurosci 8:2485–2490 1988PubMedGoogle Scholar
  14. 14.
    Guillevin L, Jarrousse B, Lok C, et al (1991) Longterm followup after treatment of polyarteritis nodosa and Churg-Strass angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange. A prospective randomized trial of 71 patients. J Rheumatol 18:567–574PubMedGoogle Scholar
  15. 15.
    Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder GG (1985) Takayasu arteritis. A study of 32 North American patients. Medicine 64(2):89–99PubMedCrossRefGoogle Scholar
  16. 16.
    Hauptman HW, Ruddy S, Roberts WN (1991) Reversal of the vasospastic component of lupus vasculopathy by infusion of prostaglandin El. J Rheumatol 18:1747–1752PubMedGoogle Scholar
  17. 17.
    Hoffman GS, Kerr GS, Leavitt RY, et al (1992) Wegener’s granulomatosis: an analysis of 158 patients. Ann Int Med 116(6):488–498PubMedCrossRefGoogle Scholar
  18. 18.
    Hoffman GS, Leavitt RY, Fleisher TA, Minor JR, Fauci AS (1990) Treatment of Wegener’s granulomatosis with intermittent high-dose intravenous cyclophosphamide. Am J Med 89(4): 403–410PubMedCrossRefGoogle Scholar
  19. Holtman JH, Neustadt DH, Klein J, Callen JP (1990) Dapsone is an effective therapy for the skin lesions of subacute cutaneous lupus erythematosus and urticarial vasculitis in a patient with C2 deficiency. J Rheumatol 17(9): 1222–1225PubMedGoogle Scholar
  20. 19.
    Hunder GG, Sheps SG, Allen GL, Joyce JW (1975) Daily and alternate-day corticosteroid regimes in treatment of giant cell arteritis. Comparison in a prospective study. Ann Int Med 82:613–618PubMedCrossRefGoogle Scholar
  21. 20.
    Isaacs JD, Clark MR, Greenwood J, Waldmann H (1992) Therapy with monoclonal antibodies. J Immunol 148:3062–3071PubMedGoogle Scholar
  22. 21.
    Jesus AD, Talal N (1990) Practical use of immunosuprressive drugs in autoimmune rheumatic diseases. Crit Care Med 18: S132–S137PubMedCrossRefGoogle Scholar
  23. 22.
    Kahan BD (1989) Drug therapy. Cyclosporine. N Engl J Med 321:1725–1736CrossRefGoogle Scholar
  24. 23.
    Kanaide H, Takeshita A, Nakamura M (1982) Etiologic aspects of coagulopathy in Takayasu’s aortitis. Am Heart J 104:1039–1045PubMedCrossRefGoogle Scholar
  25. 24.
    Kissel JT, Rammohan KW (1991) Pathogenesis and therapy of nervous system vasculitis. Clin Neuropharmacol 14:28–48PubMedCrossRefGoogle Scholar
  26. 25.
    Leavitt RY, Hoffman GS, Fauci AS (1988) The role of trimethoprim/sulfamethoxazole in the treatment of Wegener’s granulomatosis. Arthritis Rheum 31:1073–1074PubMedCrossRefGoogle Scholar
  27. 26.
    Mason WH, Jordan SC, Saki R, Takahashi M, Berstein B (1985) Circulating immune complexes in Kawasaki syndrome. Pediatr Infect Dis J 4:48CrossRefGoogle Scholar
  28. 27.
    Mathieson PW, Cobbold SP, Hale G, et al (1990) Monoclonal-antibody therapy in systemic vasculitis. N Engl J Med 323:250–254PubMedCrossRefGoogle Scholar
  29. 28.
    Mathieu A, Carcassi U (1989) Cytotoxic drugs in systemic autoimmune diseases. Clin Exp Rheumatol 7/S3:181–186Google Scholar
  30. 29.
    McCune WJ, Golbus J, Zeldes W, Böhlke P, Dunne R, Fox DA (1988) Clinical and immunologic effects of monthly administration of intravenous cyclophosphamide in severe systemic lupus erythematosus. N Engl J Med 318:1423–1431PubMedCrossRefGoogle Scholar
  31. 30.
    Moore PM, Fauci AS (1981) Neurologic manifestations of systemic vasculitis. A retrospective and prospective study of the clinicopathologic features and responses to therapy in 25 patients. Am J Med 71:517–524PubMedCrossRefGoogle Scholar
  32. 31.
    Neild GH, Ivory K, Williams DG (1984) Severe systemic vascular necrosis in cyclosporin-treatment rabbits with acute serum sickness. Br J Exp Pathol 65:731–743PubMedCentralPubMedGoogle Scholar
  33. 32.
    Patton WF, Lynch JP (1982) Lymphomatoid granulomatosis. Clinicopathologic study of four cases and literature review. Medicine 61:1–12PubMedCrossRefGoogle Scholar
  34. 33.
    Pirofsky B, Kinzey DM (1992) Intravenous immune globulins. A review of their uses in selected immunodeficiency and autoimmune diseases. Drugs 43:6–14PubMedCrossRefGoogle Scholar
  35. 34.
    Scott DGI, Bacon PA (1984) Intravenous cyclophosphamide plus methylprednisolone in treatment of systemic rheumatoid vasculitis. Am J Med 76:377–384PubMedCrossRefGoogle Scholar
  36. 35.
    Shelhamer JH, Voldman DJ, Parrillo JE, Lawley TJ, Johnston MR, Fauci AS (1985) Takayasu’s arteritis and its therapy. Ann Int Med 103:121–126PubMedCrossRefGoogle Scholar
  37. 36.
    Vincent FM (1977) Granulomatous angiitis. N Engl J Med 296:452PubMedGoogle Scholar
  38. 37.
    Yazici H, Pazarli H, Barnes CG, et al (1990) A controlled trial of azathioprine in Behget’s syndrome. N Engl J Med 322:281–285PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1993

Authors and Affiliations

  • Patricia M. Moore
  • Norbert Gretz

There are no affiliations available

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