Skip to main content
SpringerLink
Log in

Peripheral neuropathy due to vasculitis: immunopathogenesis, clinical features and treatment

  • Chapter
Immunology of Neuromuscular Disease

Part of the book series: Immunology and Medicine Series ((IMME,volume 24))

Abstract

In the 15 years that have elapsed since the publication of Fauci et al.’s seminal view on vasculitis1, there has been significant growth in our understanding of this enigmatic group of disorders. Advances in basic and clinical research have resulted in new concepts concerning the aetiology, immunology, and pathophysiology of the vasculitides, and new proposals for the diagnosis and classification of these diseases. More importantly, new and innovative therapies have been proposed based on current formulations of the immunopathogenesis of the blood vessel damage. Most of this information is immediately relevant for the practising clinician, who is faced with the formidable task of diagnosing and treating these difficult patients. This review will summarize our current understanding of vasculitis affecting the peripheral nervous system and outline the diagnosis and clinical management of these patients.

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 39.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight 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

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Fauci AS, Haynes BF, Katz P. The spectrum of vasculitis. Clinical, pathologic, immunologic, and therapeutic considerations. Ann Intern Med. 1978;89:660–76.

    PubMed  CAS  Google Scholar 

  2. Cupps TR, Fauci AS. The vasculitides. In: Smith LM, ed. Major problems in internal medicine. Vol. 21. Philadelphia: WB Saunders, 1981:6–19.

    Google Scholar 

  3. Hunder GG, Arend WP, Block DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Arthritis Rheum. 1990;33:1065–7.

    Article  PubMed  CAS  Google Scholar 

  4. Bloch DA, Michel BA, Hunder GG, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Patients and methods. Arthritis Rheum. 1990;33:1068–73.

    Article  PubMed  CAS  Google Scholar 

  5. Moore PM, Cupps TR. Neurologic complications of vasculitis. Ann Neurol. 1983;14: 155–67.

    Article  PubMed  CAS  Google Scholar 

  6. Cohen SB, Hurd ER. Neurological complications of connective tissue and other ‘collagen-vascular’ diseases. Semin Arthritis Rheum. 1981;11:190–212.

    Article  PubMed  CAS  Google Scholar 

  7. Moore PM, Fauci AS. Neurologic manifestations of systemic vasculitis. A retrospective and prospective study of the clinicopathologic features and responses to therapy in 25 patients. Am J Med. 1981;71:517–24.

    Article  PubMed  CAS  Google Scholar 

  8. Sigal LH. The neurologic presentation of vasculitis and rheumatologic syndromes. Medicine. 1987;66:157–80.

    Article  PubMed  CAS  Google Scholar 

  9. Kissel JT, Slivka AP, Warmolts JR, et al. The clinical spectrum of necrotizing angiopathy of the peripheral nervous system. Ann Neurol. 1985;18:251–7.

    Article  PubMed  CAS  Google Scholar 

  10. Dyck PJ, Benstead TJ, Conn DL, et al. Nonsystemic vasculitic neuropathy. Brain. 1987;110:843–54.

    Article  PubMed  Google Scholar 

  11. Hawke SHB, Davies L, Pamphlett R, Guo Y-P, Pollard JD, McLeod JG. Vasculitic neuropathy. A clinical and pathological study. Brain. 1991;114:2175–90.

    Article  PubMed  Google Scholar 

  12. Smiley JD, Moore M. Immune complex vasculitis: role of complement and IgG-Fc receptor functions. Am J Med Sci. 1989;298:267–77.

    Article  PubMed  CAS  Google Scholar 

  13. McDougal JS, McDuffie FC. Immune complexes in man: detection and clinical significance. Adv Clin Chem. 1985;24:1–60.

    Article  PubMed  CAS  Google Scholar 

  14. Cornacoff JB, Hebert LA, Smead WL, et al. Primate erythrocyte-immune complex-clearing mechanism. J Clin Invest. 1983;71:236–47.

    Article  PubMed  CAS  Google Scholar 

  15. Becherer JD, Alsenz J, Servic C, et al. Cell surface proteins reacting with activated complement components. Complement Inflamm. 1989;6:142–65.

    PubMed  CAS  Google Scholar 

  16. Fauci AS. The vasculitic syndromes. In: Wilson JD, Braunwald E, Issellbacher KJ, et al. (eds). Harrison’s Principles of Internal Medicine. New York: McGraw Hill, 1991;1456–63.

    Google Scholar 

  17. Savage COS, Ng YC. The aetiology and pathogenesis of major systemic vasculitides. Postgrad Med J. 1986;62:627–36.

    Article  PubMed  CAS  Google Scholar 

  18. Hogg N. The structure and function of Fe receptors. Immunol Today. 1988;9:185–7.

    Article  PubMed  CAS  Google Scholar 

  19. Moore PM. Immune mechanisms in the primary and secondary vasculitides. J Neurol Sci. 1989;93:129–45.

    Article  PubMed  CAS  Google Scholar 

  20. Kissel JT, Riethman JL, Omerza J, et al. Peripheral nerve vasculitis: immune characterization of the vascular lesions. Ann Neurol. 1989;25:291–7.

    Article  PubMed  CAS  Google Scholar 

  21. Wiedmer T, Esmon CT, Sims PJ. Complement proteins C5b-9 stimulate procoagulant activity through platelet prothrombinase. Blood. 1986;4:875–80.

    Google Scholar 

  22. Ryan US. The endothelial surface and responses to injury. Fed Proc. 1986;45:101–8.

    PubMed  CAS  Google Scholar 

  23. Pangyres PK, Blumbergs PC, Leong AS-Y, Bourne AJ. Vasculitis of peripheral nerve and skeletal muscle: clinicopathological correlation and immunopathic mechanisms. J Neurol Sci. 1990;100:193–202.

    Article  Google Scholar 

  24. Pober JS, Cotran R. Cytokines and endothelial cell biology. Physiol Rev. 1990;70:427–51.

    PubMed  CAS  Google Scholar 

  25. Cavender DE. Organ-specific and non-organ-specific lymphocyte receptors for vascular endothelium. J Invest Dermatol. 1990;94(suppl.):41–85.

    Article  Google Scholar 

  26. Brasile L, Kremer JM, Clarke JL. Identification of an autoantibody to vascular endothelial cell-specific antigens in patients with systemic vasculitis. Am J Med. 1989;87:74–80.

    Article  PubMed  CAS  Google Scholar 

  27. Frampton G, Jayne DRW, Perry GJ et al. Autoantibodies to endothelial cells and neutrophil cytoplasmic antigens in systemic vasculitis. Clin Exp Immunol. 1990;82:227–32.

    Article  PubMed  CAS  Google Scholar 

  28. Editorial. Antibodies to endothelial cells. Lancet. 1991;337:649–50.

    Article  Google Scholar 

  29. Gaskin G, Savage COS. Immune markers in vasculitis. Br J Hosp Med. 1991;46:104–6.

    PubMed  CAS  Google Scholar 

  30. Ferraro G, Meroni PL, Tincani A et al. Anti-endothelial cell antibodies in patients with Wegener’s granulomatosis and micropolyarteritis. Clin Exp Immunol. 1990;79:47–53.

    Article  PubMed  CAS  Google Scholar 

  31. Goeken JA. Antineutrophil cytoplasmic antibody - a useful serological marker for vasculitis. 7 Clin Immunol. 1991;11:161–74.

    CAS  Google Scholar 

  32. Parlevliet KJ, Henzen-Logmans SC, Oe PL, et al. Antibodies to components of neutrophil cytoplasm: a new diagnostic tool in patients with Wegener’s granulomatosis and systemic vasculitis. Q J Med. 1988;66:55–63.

    PubMed  CAS  Google Scholar 

  33. Egner W, Chapel HM. Titration of antibodies against neutrophil cytoplasmic antigens is useful in monitoring disease activity in systemic vasculitides. Clin Exp Immunol. 1990;82: 244–9.

    Article  PubMed  CAS  Google Scholar 

  34. Lai KN, Jayne DRW, Brownlee A, Lockwood CM. The specificity of anti-neutrophil cytoplasm antibodies in systemic vasculitides. Clin Exp Immunol. 1990;82:233–7.

    Article  PubMed  CAS  Google Scholar 

  35. Bleil L, Manger B, Winkler TH, et al. The role of antineutrophil cytoplasm antibodies, anticardiolipin antibodies, von Willebrand factor antigen, and fibronectin for the diagnosis of systemic vasculitis. J Rheumatol. 1991;18:1199–206.

    PubMed  CAS  Google Scholar 

  36. Ewert BH, Jennette JC, Falk RJ. The pathogenic role of antineutrophil cytoplasmic autoantibodies. Am J Kidney Dis. 1991;18:188–95.

    PubMed  CAS  Google Scholar 

  37. Nolte B, Specks U, Liidemann J et al. Anticytoplasmic antibodies: their immunodiagnostic value in Wegener’s granulomatosis. Ann Intern Med. 1989;111:28–40.

    Google Scholar 

  38. Panegyres PK, Faull RJ, Russ GR, Appleton SL, Wangel AG, Blumbergs PC. Endothelial cell activation in vasculitis of peripheral nerve and skeletal muscle. J Neurol Neurosurg Psychiatry. 1992;55:4–7.

    Article  PubMed  CAS  Google Scholar 

  39. Shuki H, Shimokama T, Watanabe T. Temporal arteritis. Cell composition and the possible pathogenetic role of cell-mediated immunity. Hum Pathol. 1989;20:1057–64.

    Article  Google Scholar 

  40. Gephardt GN, Ahmad M, Tubbs RR. Pulmonary vasculitis (Wegener’s granulomatosis). Immunohistochemical study of T and B cell markers. Am J Med. 1983;74:700–4.

    Article  PubMed  CAS  Google Scholar 

  41. Grau GE, Roux-Lombard P, Gysler C et al. Serum cytokine changes in systemic vasculitis. Immunology. 1989;68:196–8.

    PubMed  CAS  Google Scholar 

  42. Grau GE, Piguet PF, Vassalli P, Lambert PH. Involvement of tumour necrosis factor and other cytokines in immune-mediated vascular pathology. Int Arch Allergy Appl Immunol. 1989;88:34–9.

    Article  PubMed  CAS  Google Scholar 

  43. Braquet P, Hosford D, Braquet M, et al. Role of cytokines and platelet-activating factor in microvascular immune injury. Int Arch Allergy Appl Immunol. 1989;88:88–100.

    Article  PubMed  CAS  Google Scholar 

  44. Mancardi GL, Cadoni A, Zicca A et al. HLA-DR Schwann cell reactivity in peripheral neuropathies of different origins. Neurology. 1988;38:848–51.

    Article  PubMed  CAS  Google Scholar 

  45. Nukada H, Dyck PJ. Acute ischemia causes axonal stasis, swelling, attenuation and secondary demyelination. Ann Neurol. 1987;22:311–18.

    Article  PubMed  CAS  Google Scholar 

  46. Said G, Laxroix-Ciaudo C, Fujimura H et al. The peripheral neuropathy of necrotizing arteritis: a clinicopathological study. Ann Neurol. 1988;23:461–5.

    Article  PubMed  CAS  Google Scholar 

  47. Fujimura H, Lacroix C, Said G. Vulnerability of nerve fibers to ischaemia. A quantitative light and electron microscope study. Brain. 1991;114:1929–42.

    Article  PubMed  Google Scholar 

  48. Dyck P, Conn DL, Okazaki H. Necrotizing angiopathic neuropathy: three-dimensional morphology of fiber degeneration related to sites of occluded vessels. Mayo Clin Proc. 1972;47:461–75.

    PubMed  CAS  Google Scholar 

  49. Wees SJ, Sunwoo IN, Oh SJ. Sural nerve biopsy in systemic necrotizing vasculitis. Am J Med. 1981;71:525–32.

    CAS  Google Scholar 

  50. Harati Y, Niakan E. The clinical spectrum of inflammatory-angiopathic neuropathy. J Neurol Neurosurg Psychiatry. 1986;49:1313–16.

    Article  CAS  Google Scholar 

  51. Bouche P, Leger JM, Travers MA et al Peripheral neuropathy in systemic vasculitis: clinical and electrophysiologic study of 22 patients. Neurology. 1986;36:1598–602.

    Article  PubMed  CAS  Google Scholar 

  52. Olney RK. Neuropathies in connective tissue disease. Muscle Nerve. 1992;15:531–42.

    Article  PubMed  CAS  Google Scholar 

  53. Ropert A, Metral S. Conduction block in neuropathies with necrotizing vasculitis. Muscle Nerve. 1990;13:102–5.

    Article  PubMed  CAS  Google Scholar 

  54. Jamieson PW, Guiliani MJ, Martinez AJ. Necrotizing angiopathy presenting with multifocal conduction blocks. Neurology. 1991;41:442–4.

    Article  PubMed  CAS  Google Scholar 

  55. Kissel JT, Mendell JR. Vasculitic neuropathy. Neurol Clin. 1992;10:761–81.

    PubMed  CAS  Google Scholar 

  56. Scott DGI. Classification and treatment of systemic vasculitis. Br J Rheumatol. 1988;27: 251–7.

    Article  PubMed  CAS  Google Scholar 

  57. Haynes BF, Allen NB, Fauci AS. Diagnostic and therapeutic approach to the patient with vasculitis. Med Clin North Am. 1986;70:355–68.

    PubMed  CAS  Google Scholar 

  58. Axelrod L. Glucocorticoids. In: Kelley WN, Harris ED, Ruddy S et al., editors. Textbook of Rheumatology. New York: WB Saunders, 1989;845–61.

    Google Scholar 

  59. Dalakas M. Pharmacologic concerns of corticosteroids in the treatment of patients with immune-related neuromuscular diseases. Neurol Clin. 1990;8:93–118.

    PubMed  CAS  Google Scholar 

  60. Mankin HJ. Nontraumatic necrosis of bone (osteonecrosis). N Eng] J Med. 1992;326: 1473–9.

    Article  CAS  Google Scholar 

  61. Stillwell TJ, Benson RC, DeRemee RA et al. Cyclophosphamide-induced bladder toxicity in Wegener’s granulomatosis. Arthritis Rheum. 1988;31:465–70.

    Article  PubMed  CAS  Google Scholar 

  62. Katz P, Fauci AS. Immunosuppressives and immunoadjuvants. In: Samter M, Talmage DW, Frank MM et al., editors, Immunological Disease. Boston: Little Brown, 1988: 675–98.

    Google Scholar 

  63. Kisel JT, Levy RJ, Mendell JR, Griggs RC. Azathioprine toxicity in neuromuscular disease. Neurology. 1986;36:35–9.

    Article  Google Scholar 

  64. Yazici H, Pazarli H, Barnes CG et al. A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med. 1990;322:281–5.

    Article  PubMed  CAS  Google Scholar 

  65. Kissel JT, Rammohan KW. Pathogenesis and therapy of nervous system vasculitis. Clin Neuropharmacol. 1991;14:28–48.

    Article  PubMed  CAS  Google Scholar 

  66. Fauci AS, Katz P, Haynes BF, et al. Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med. 1979;301:235–8.

    Article  PubMed  CAS  Google Scholar 

  67. Leib ES, Restivo C, Paulus HE Immunosuppressive and corticosteroid therapy of polyarteritis nodosa. Am J Med. 1979;67:941–7.

    Article  PubMed  CAS  Google Scholar 

  68. Fauci AS, Haynes BF, Katz P et al. Wegener’s granulomatosis: prospective and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98:76–85.

    PubMed  CAS  Google Scholar 

  69. Ballow M. Mechanisms of action of intravenous immunoglobulin therapy and potential use in autoimmune connective tissue diseases. Cancer. 1991;68:1430–6.

    Article  PubMed  CAS  Google Scholar 

  70. Schwartz SA. Intravenous immunoglobulin (IVIG) in the therapy of autoimmune disorders. J Clin Immunol. 1990;10:81–9.

    Article  PubMed  CAS  Google Scholar 

  71. Dwyer JM. Manipulating the immune system with immune globulin. N Engl J Med. 1992;326:107–16.

    Article  PubMed  CAS  Google Scholar 

  72. Jaynes DRW, Davies MJ, Fox CJV, Black CM, Lockwood CM. Treatment of systemic vasculitis with pooled intravenous immunoglobulin. Lancet. 1991;337:1137–9.

    Article  Google Scholar 

  73. Conn DL. Update on systemic necrotizing vasculitis. Mayo Clin Proc. 1989;64:535–43.

    PubMed  CAS  Google Scholar 

  74. Huse WD, Lakshmi S, Iverson SA et al. Generation of a large combinatorial library of the immunoglobulin repertoire in phage lambda. Science. 1989;246:1275–81.

    Article  PubMed  CAS  Google Scholar 

  75. Mathieson PW, Cobbold SP, Hale G et al. Monoclonal antibody therapy in systemic vasculitis. N Engl J Med. 1990;323:250–4.

    Article  PubMed  CAS  Google Scholar 

Download references

Authors
  1. J. T. Kissel
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. J. R. Mendell
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Department of Neurology, Klinikum Großhadern, Ludwig-Maximilians University, Munich, Germany

    R. Hohlfeld

  2. Department of Neuroimmunology, Max-Planck Institute, Martinsried, Germany

    R. Hohlfeld

Rights and permissions

Reprints and permissions

Copyright information

© 1994 Springer Science+Business Media Dordrecht

About this chapter

Cite this chapter

Kissel, J.T., Mendell, J.R. (1994). Peripheral neuropathy due to vasculitis: immunopathogenesis, clinical features and treatment. In: Hohlfeld, R. (eds) Immunology of Neuromuscular Disease. Immunology and Medicine Series, vol 24. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-1422-6_4

Download citation

  • DOI: https://doi.org/10.1007/978-94-011-1422-6_4

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-4622-0

  • Online ISBN: 978-94-011-1422-6

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation