Skip to main content

Advertisement

Log in

Primary and secondary vasculitic neuropathy

  • REVIEW
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Necrotizing vasculitis occurs as a primary phenomenon in connective tissue disorders and cognate fields, including polyarteritis nodosa and the Churg and Strauss syndrome variant, rheumatoid arthritis, systemic lupus and Wegener’s granulomatosis. In all these conditions focal and multifocal neuropathy occur as a consequence of destruction of the arterial wall and occlusion of the lumen of small epineurial arteries. Vasculitis may also complicate the course of other conditions ranging from infection with the HIV and with the B and C hepatitis viruses to diabetes and sarcoidosis. Pathologically polymorphonuclear cells are present in the infiltrates of the vessel wall in primary necrotizing vasculitis, while in secondary vasculitis the inflammatory infiltrate is mainly composed of mononuclear cells. In all instances symptomatic vasculitis requires corticosteroid to control the inflammatory process and prevent further ischemic nerve lesions.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Asbury AK, Johnson PC (1978) Vasculitic neuropathy. In: Bennington JL (ed) Major Problems in Pathology (Vol 9, Pathology of Peripheral Nerve) Philadelphia, Saunders, pp 110–119

  2. Bonnin H, Moretti G-F, Rivière J (1948) La biopsie musculaire révélatrice des formes non cutanées de la périartérite noueuse. J Med Bordeaux 125:303–308

    Google Scholar 

  3. Bouche P, Léger JM, Travers MA, Cathala HP, Castaigne P (1986) Peripheral neuropathy in systemic vasculitis: Clinical and electrophysiological study of 22 patients. Neurology 36:1598–1602

    CAS  PubMed  Google Scholar 

  4. Calabrese LH, Estes M, Yen-Lieberman B, Proffitt MR, Tubbs R, Fishleder AJ, Levin KH (1989) Systemic vasculitis in association with human immunodeficiency virus infection. Arthr Rheum 32:569–576

    CAS  Google Scholar 

  5. Chia L, Fernandez A, Lacroix C, Adams D, Planté V, Said G (1996) Contribution of nerve biopsy findings to the diagnosis of disabling neuropathy in the elderly: a retrospective review of 100 consecutive patients. Brain 119:1091–1098

    PubMed  Google Scholar 

  6. Chumbley LC, Harrison EG Jr, Deremee RA (1977) Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Proc Mayo Clin 52:477

    CAS  PubMed  Google Scholar 

  7. Churg J, Strauss L (1951) Allergic granulomatosis, allergic angeitis, and periarteritis nodosa. Am J Pathol 27:277–301

    CAS  PubMed  Google Scholar 

  8. Cohen–Tervaert JW, Limburg PC, Elema JD, et al. (1991). Detection of autoantibodies against myeloid lysosomal enzymes: a useful adjunct to classification of patients with biopsyproven necrotising arteritis. Am J Med 91:1991

    Google Scholar 

  9. Collins MP, Periquet I (2002) Prevalence of vasculitic neuropathy in Wegener granulomatosis. Arch Neurol 59:1333–1334

    Article  PubMed  Google Scholar 

  10. Cornblath DR, Sumner AJ (1991) Conduction block in neuropathies with necrotizing vasculitis (letter). Muscle Nerve 14:185

    Article  CAS  PubMed  Google Scholar 

  11. De Groot K, Schmidt DK, Arlt AC, Gross WL, Reinhold-Keller E (2001) Standardized neurologic evaluation of 128 patients with Wegener granulomatosis. Arch Neurol 58:1215–1221

    Article  CAS  PubMed  Google Scholar 

  12. Diaz-Perez J, Winkelmann RK (1980) Cutaneous periarteritis nodosa: a study of 33 cases. In:Wolff K, Winkelmann RK (eds) London, Lloyd-Luke Ltd, pp 273–284

  13. Drüeke T, Barbanel C, Jungers P, Digeon M, Poisson M, Brivet, Trecan G, Feldmann G, Crosnier J, Bach JF (1980) Hepatitis B antigen associated periarteritis nodosa in patients undergoing long-term hemodialysis. Am J Med 68:86–90

    Article  PubMed  Google Scholar 

  14. Dyck PJ, Benstead TJ, Conn DL, Stevens JC, Windebank AJ, Low PA (1987) Nonsystemic vasculitic neuropathy. Brain 110:843–854

    PubMed  Google Scholar 

  15. Dyck PJB, Norell JE, Dyck PJ (1999) Microvasculitis and ischemia in diabetic lumbosacral radiculoplexus neuropathy. Neurology 53:2113–2121

    CAS  PubMed  Google Scholar 

  16. Ewert BH, Jennette JC, Falk RJ (1991) The pathogenetic role of antineutrophil cytoplasmic antibodies. Am J Kidney Dis 18:188–195

    CAS  PubMed  Google Scholar 

  17. Fauci AS, Haynes BF, Katz P (1978) The spectrum of vasculitis. Ann Int Med 9:660–676

    Google Scholar 

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

    CAS  PubMed  Google Scholar 

  19. Frampton G, Jayne DRW, Perry GJ, et al. (1990) Autoantibodies to endothelial cells and neutrophil cytoplasmic antigens in systemic vasculitis. Clin Exp Immunol 79:47–53

    CAS  PubMed  Google Scholar 

  20. Frohnert PP, Sheps SG (1967) Longterm follow up study of periarteritis nodosa. Am J Med 43:8–13

    Article  CAS  PubMed  Google Scholar 

  21. Fujimura H, Lacroix C, Said G (1991) Vulnerability of nerve fibers to ischaemia. Brain 114:1929–1942

    PubMed  Google Scholar 

  22. Godeau P, Guillevin L (1982) Périartérite noueuse systémique. In: Kahn M-F, Peltier AP (eds) Maladies dites systémiques. Flammarion, Paris, pp 414–445

  23. Goeken JA (1991) Antineutrophil cytoplasmic antibody – a useful serological marker for vasculitis. J Clin Immunol 11:161–174

    Article  CAS  PubMed  Google Scholar 

  24. Harati Y, Niakan A (1986) The clinical spectrum of inflammatory angiopathic neuropathy. J Neurol Neurosurg Psychiatry 49:1313–1316

    CAS  PubMed  Google Scholar 

  25. Hattori N, Ichimura M, Nagamatsu M, Li M, Yamamoto K, Kumazawa K (1999) Clinicopathological Features Of Churg-Strauss Syndrome Associated Neuropathy. Brain 122:427–439

    Article  PubMed  Google Scholar 

  26. Hawke SHB, Davies L, Pamphlett R, Guo YP, Pollard JD, McLeod JG (1991) Vasculitic neuropathy. A clinical and pathological study. Brain 114:2175–2190

    PubMed  Google Scholar 

  27. Hogg N (1988) The structure and function of Fc receptors. Immunol Today 9:185–187

    Article  CAS  PubMed  Google Scholar 

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

    CAS  Google Scholar 

  29. Kissel JT, Riethman JL, Ormeza J, Rammohan KW, Mendell JR (1989) Peripheral nerve vasculitis: immune characterization of the vascular lesions. Ann Neurol 93:129–145

    Google Scholar 

  30. Kissel JT, Mendell JR (1994) Peripheral neuropathy due to vasculitis: immunopathogenesis, clinical features and treatment. In: R Hohlfeld (ed) Immunology of Neuromuscular Disease. Kluwer Academic Press, Dordrecht, pp 105–121

  31. Kussmaul A, Maier R (1866) Über eine bisher nicht beschriebene eigenthümliche Arterienerkrankung (Periarteritis nodosa), die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht. Dtsch Arch Klin Med 1:484–517

    Google Scholar 

  32. Lacroix C, Lozeron P, Ferreira A, Said G (2003) Peripheral neuropathy due to necrotizing arteritis (NA) in non-connective tissue disorders (NCTD): A clinicopathological study. J Neurol 250(Suppl 2):25

    Google Scholar 

  33. Lidove O, Maisonobe T, Servan J, Thibault V, Leger JM, Piette JC, Cacoub P (2001) Peripheral neuropathy and hepatitis C virus infection: more than cryoglobulinemia. Rev Med Interne 22:939–947

    CAS  PubMed  Google Scholar 

  34. Lie JT (1994) Nomenclature and classification of vasculitis: Plus ça change, plus c’est la même chose. Arthritis Rheum 2:181–186

    Google Scholar 

  35. Lovshin Ll, Kernohan JW (1948) Peripheral neuritis in periarteritis nodosa. Arch Intern Med 82:321–338

    Google Scholar 

  36. Maxeiner SR, MCDonald JR, Kirlklin JW (1952) Muscle biopsy in the diagnosis of periarteritis nodosa: an evaluation. Surg Clin North Am 32:1225–1235

    Google Scholar 

  37. Meyer J, Foley J, Campagna-Pinto D (1953) Granulomatous angiitis of the meninges in sarcoidosis. Arch Neurol Psychiatr 69:587

    CAS  Google Scholar 

  38. Molina R, Provost TT, Alexander EL (1985) Peripheral inflammatory vascular disease in Sjögren’s syndrome. Arthritis and Rheumatism 28:1341–1347

    CAS  PubMed  Google Scholar 

  39. Moore PM (1989) Immune mechanisms in the primary and secondary vasculitides. J Neurol Sci 93:129–145

    Article  CAS  PubMed  Google Scholar 

  40. Moore PM, Cupps TR (1983) Neurologic complications of vasculitis. Ann Neurol 14:155–167

    Article  CAS  PubMed  Google Scholar 

  41. Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE (1993) Neurological involvement in Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol 33:4–9

    Article  CAS  PubMed  Google Scholar 

  42. Nydegger UE (1985) A place for soluble immune complexes in clinical immunology? Immunology Today 6:80–82

    Article  Google Scholar 

  43. Oh SJ (1980) Sarcoid polyneuropathy: a histologically proved case. Ann Neurol 7:178–181

    Article  CAS  PubMed  Google Scholar 

  44. Pages M, Pages AM (1984) La biopsie nerveuse au cours de la périartérite noueuse. Son intérêt diagnostique. Sem Hop Paris 60:3295–3299

    Google Scholar 

  45. Panegyres PK, Blumbergs PC, Leong AS-Y, Bourne AJ (1990) Vasculitis of peripheral nerve and skeletal muscle: clinicopathological and immunopathic mechanisms. J Neurol Sci 100:193–202

    Article  CAS  PubMed  Google Scholar 

  46. Panegyres PK, Faull RJ, Russ GR, et al. (1992) Endothelial cell activation in vasculitis of peripheral nerve and skeletal muscles. J Neurol Neurosurg Psychiatry 55:4–7

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  48. Parry GJ, Brown MJ, Asbury AK (1981) Diagnostic value of nerve biopsy in mononeuritis multiplex. Neurology 31:129–130

    Google Scholar 

  49. Pleasure D (2001) Peripheral neuropathy as the first manifestation of Wegener granulomatosis. Arch Neurol 58:1204

    Article  CAS  PubMed  Google Scholar 

  50. Pober JS, Cotran RS (1990) Cytokines and endothelial cell biology. Physiol Rev 70:427–451

    CAS  PubMed  Google Scholar 

  51. Puechal X, Said G, Hilliquin P, et al. (1995) Peripheral neuropathy with necrotizing vasculitis in rheumatoid arthritis. A clinicopathologic and prognostic study of thirty two patients. Arthr Rheum 38:1618–1629

    CAS  Google Scholar 

  52. Ropert A, Metral S (1990) Conduction block in neuropathies with necrotizing arteritis. Muscle Nerve 13:102–105

    Article  CAS  PubMed  Google Scholar 

  53. Said G (1988) Vasculitic Neuropathy in the Elderly, in Peripheral Nerve Changes in the Elderly. New Issues Neurosci 1:199–204

    Google Scholar 

  54. Said G, Lacroix C, Fujimura H, Blas C, Faux N (1988) The peripheral neuropathy of necrotizing arteritis: a clinicopathologic study. Ann Neurol 23:461–465

    Article  CAS  PubMed  Google Scholar 

  55. Said G, Goulon-Goeau C, Lacroix C, Moulonguet A (1994) Proximal Diabetic Neuropathy – Clinical Aspects and Morphogical Findings in Biopsy Specimens of the Intermediate Cutaneous Nerve of the Thigh. Ann Neurol 35:559–569

    Article  CAS  PubMed  Google Scholar 

  56. Said G, Lacroix C, Planté-Bordeneuve V, Le Page L, Pico F, Presles O, Senant J, Remy P, Rondepierre P, Mallecourt J (2002) Nerve granulomas and vasculitis in sarcoid peripheral neuropathy: a clinicopathological study of 11 patients. Brain 125:264–275

    Article  PubMed  Google Scholar 

  57. Said G, Lacroix C, Lozeron P, Guglielmi J-M, Ropert A, Planté V, Adams D (2003) Inflammatory vasculopathy in multifocal diabetic neuropathy. Brain 126:376–385

    Article  PubMed  Google Scholar 

  58. Smiley JD, Moore SE (1989) Immune complex vasculitis: role of complement and IgG-Fc receptor functions. Am J Med Sci 298:267–297

    CAS  PubMed  Google Scholar 

  59. Stern GM, Hoffbrand AV, Urich H (1965) The peripheral nerves and skeletal muscles in Wegener’s granulomatosis: clinicopathological study of four cases. Brain 88:151–164

    CAS  PubMed  Google Scholar 

  60. Torvik A, Berntzen A (1968) Necrotizing vasculitis without visceral involvement. Acta med Scand 184:69–77

    CAS  PubMed  Google Scholar 

  61. Vital C, Aubertin, Ragnault J, Amigues H, Mouton L, Bellance R (1982) Sarcoidosis of the peripheral nerve: a histological and ultrastructural study of two cases. Acta Neuropathol (Berl) 58:111–114

    Article  CAS  PubMed  Google Scholar 

  62. Wattiaux MJ, Kahn MF, Thevenet JP, Sauvezie B, Imbert JC (1987) L’atteinte vasculaire de la polyarthrite rhumatoïde: étude rétrospective de 37 polyarthrites rhumatoïdes avec atteinte vasculaire et revue de la littérature. Ann Med Int 138:566–587

    CAS  Google Scholar 

  63. Wees SJ, Sunwood LN, Oh SJ (1981) Sural nerve biopsy in systemic necrotizing vasculitis. Am J Med 71:525–532

    Article  CAS  PubMed  Google Scholar 

  64. Winkelmann RK (1980) Classification of Vasculitis. In:Wolff K, Winkelmann RK (eds) Lloyd-Luke Ltd, London, pp 1–24

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to G. Said.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Said, ., Lacroix, C. Primary and secondary vasculitic neuropathy. J Neurol 252, 633–641 (2005). https://doi.org/10.1007/s00415-005-0833-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-005-0833-9

Key words

Navigation