Clinical manifestations of vasculitic neuropathy
- Steven David Brass, MD, MPH, MBA
Steven David Brass, MD, MPH, MBA
- Director of Medical Affairs
- Providence Little Company of Mary Medical Center
- Simon M Helfgott, MD
Simon M Helfgott, MD
- Associate Professor of Medicine
- Harvard Medical School
- Section Editors
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
Vasculitic diseases can affect any organ or tissue, including the peripheral nerves. When vasculitis affects the vasa nervorum, the results are often critical ischemia to peripheral nerves and potentially profound clinical sequelae.
Vasculitic neuropathy is usually only one feature of a systemic condition that can involve the skin, lungs, kidneys, and other organs. As an example, the group of vasculitic disorders commonly associated with antineutrophil cytoplasmic antibodies (ANCA; ie, granulomatosis with polyangiitis [GPA; Wegener’s], microscopic polyangiitis [MPA], and eosinophilic granulomatosis with polyangiitis [EGPA; Churg-Strauss]) often cause vasculitic neuropathy. Polyarteritis nodosa (PAN) and mixed cryoglobulinemia are also prone to involve the peripheral nervous system.
In a minority of patients with vasculitic neuropathy, the peripheral nervous system vasculitis occurs in isolation. In this setting, the disorder is referred to as nonsystemic vasculitic neuropathy or isolated peripheral nervous system vasculitis [1-4].
Even in the absence of frank systemic vasculitis, nonsystemic vasculitic neuropathy can be a devastating illness, leading to severe functional disabilities from which some patients never recover fully. Because the regeneration of peripheral nerves may require months or years, the goal of therapy is to control the inflammation within the vasa nervorum as quickly as possible to prevent or minimize nerve damage.
This topic will review the clinical manifestations of vasculitic peripheral neuropathy. The diagnosis and treatment of vasculitic neuropathy are presented separately. (See "Diagnosis and treatment of vasculitic neuropathy".)
- Dyck PJ, Benstead TJ, Conn DL, et al. Nonsystemic vasculitic neuropathy. Brain 1987; 110 ( Pt 4):843.
- Collins MP, Periquet MI. Non-systemic vasculitic neuropathy. Curr Opin Neurol 2004; 17:587.
- Davies L, Spies JM, Pollard JD, McLeod JG. Vasculitis confined to peripheral nerves. Brain 1996; 119 ( Pt 5):1441.
- Mathew L, Talbot K, Love S, et al. Treatment of vasculitic peripheral neuropathy: a retrospective analysis of outcome. QJM 2007; 100:41.
- Ransohoff R, Beneviste E, Cadavid D. Vascultis and vasculopathies of the nervous system. In: Continiuum neuroimmunology, First Edition, Mancall EL, Cascino TL, Devereaux MW, Lambert AL (Eds), Lippincott Williams & Wilkins, Philadelphia 2001. p.146.
- Hawke SH, Davies L, Pamphlett R, et al. Vasculitic neuropathy. A clinical and pathological study. Brain 1991; 114 ( Pt 5):2175.
- Saadoun D, Bieche I, Maisonobe T, et al. Involvement of chemokines and type 1 cytokines in the pathogenesis of hepatitis C virus-associated mixed cryoglobulinemia vasculitis neuropathy. Arthritis Rheum 2005; 52:2917.
- Broglio L, Erne B, Tolnay M, et al. Allograft inflammatory factor-1: a pathogenetic factor for vasculitic neuropathy. Muscle Nerve 2008; 38:1272.
- Said G, Lacroix C. Primary and secondary vasculitic neuropathy. J Neurol 2005; 252:633.
- Lie JT. Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am 1995; 21:883.
- Steck AJ, Kinter J, Renaud S. Differential gene expression in nerve biopsies of inflammatory neuropathies. J Peripher Nerv Syst 2011; 16 Suppl 1:30.
- Kinter J, Broglio L, Steck AJ, et al. Gene expression profiling in nerve biopsy of vasculitic neuropathy. J Neuroimmunol 2010; 225:184.
- Allan SG, Towla HM, Smith CC, et al. Painful brachial plexopathy: an unusual presentation of polyarteritis nodosa. Postgrad Med J 1982; 58:311.
- Pagnoux C, Guillevin L. Peripheral neuropathy in systemic vasculitides. Curr Opin Rheumatol 2005; 17:41.
- Suppiah R, Hadden RD, Batra R, et al. Peripheral neuropathy in ANCA-associated vasculitis: outcomes from the European Vasculitis Study Group trials. Rheumatology (Oxford) 2011; 50:2214.
- Samson M, Puéchal X, Devilliers H, et al. Mononeuritis multiplex predicts the need for immunosuppressive or immunomodulatory drugs for EGPA, PAN and MPA patients without poor-prognosis factors. Autoimmun Rev 2014; 13:945.
- Garcia-Bragado F, Fernandez JM, Navarro C, et al. Peripheral neuropathy in essential mixed cryoglobulinemia. Arch Neurol 1988; 45:1210.
- Gemignani F, Melli G, Inglese C, Marbini A. Cryoglobulinemia is a frequent cause of peripheral neuropathy in undiagnosed referral patients. J Peripher Nerv Syst 2002; 7:59.
- Gemignani F, Brindani F, Alfieri S, et al. Clinical spectrum of cryoglobulinaemic neuropathy. J Neurol Neurosurg Psychiatry 2005; 76:1410.
- Griffin JW. Vasculitic neuropathies. Rheum Dis Clin North Am 2001; 27:751.
- Said G. Vasculitic neuropathy. Curr Opin Neurol 1999; 12:627.
- McCombe PA, McLeod JG, Pollard JD, et al. Peripheral sensorimotor and autonomic neuropathy associated with systemic lupus erythematosus. Clinical, pathological and immunological features. Brain 1987; 110 ( Pt 2):533.
- McNicholl JM, Glynn D, Mongey AB, et al. A prospective study of neurophysiologic, neurologic and immunologic abnormalities in systemic lupus erythematosus. J Rheumatol 1994; 21:1061.
- Omdal R, Mellgren SI, Husby G, et al. A controlled study of peripheral neuropathy in systemic lupus erythematosus. Acta Neurol Scand 1993; 88:41.
- Ramos-Casals M, Nardi N, Lagrutta M, et al. Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients. Medicine (Baltimore) 2006; 85:95.
- Puéchal X, Said G, Hilliquin P, et al. Peripheral neuropathy with necrotizing vasculitis in rheumatoid arthritis. A clinicopathologic and prognostic study of thirty-two patients. Arthritis Rheum 1995; 38:1618.
- Kararizou E, Davaki P, Karandreas N, et al. Nonsystemic vasculitic neuropathy: a clinicopathological study of 22 cases. J Rheumatol 2005; 32:853.
- Collins MP, Dyck PJ, Gronseth GS, et al. Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst 2010; 15:176.
- Patterns of nerve involvement
- CLINICAL MANIFESTATIONS
- Neurologic features
- - Mononeuritis multiplex
- - Polyneuropathy
- - Radiculopathy and/or plexopathy
- Non-neurologic features
- SYSTEMIC DISEASE ASSOCIATIONS
- Systemic vasculitis
- - ANCA-associated vasculitis
- - Polyarteritis nodosa
- - Cryoglobulinemic vasculitis
- Connective tissue diseases
- - Lupus
- - Rheumatoid arthritis
- - Sjögren's syndrome
- NONSYSTEMIC VASCULITIC NEUROPATHY