Vasculitic Neuropathy Treatment & Management
- Author: Abbas Mehdi, MD; Chief Editor: Nicholas Lorenzo, MD more...
Medical Care
Early use of an aggressive immunosuppressive agent is indicated to treat vasculitic neuropathy.
Combination therapy with corticosteroids and cyclophosphamide is reported to be more effective than monotherapy in inducing remission and improving disability and in reducing relapse rate and chronic pain.
One cohort study shows a 61% response rate with a 59% relapse rate with corticosteroid monotherapy compared with a 95% response rate with a 29% relapse rate in combination therapy of corticosteroids and cyclophosphamide.
- Treat systemic necrotizing vasculitis and nonsystemic vasculitis with a combination of prednisone and cyclophosphamide or, less commonly, azathioprine (Imuran), methotrexate, and intravenous immunoglobulin.
- Administer prednisone for 2-3 months or until clinical beneficial effect is noted; then taper gradually according to the patient's response. The typical starting dose is 40-100 mg/d, which is eventually tapered to every other day dosing to avoid side effects. Cyclophosphamide is started at 100-150 mg/d.
- Continue treatment for 6 months to 1 year or longer. Some patients may require long-term immunosuppression for years.
- Discuss adverse affects and toxicity of long-term use of corticosteroids and immunosuppressive agents with the patient and family prior to onset of therapy.
- Symptomatic treatment of neuropathic pain can be initiated with amitriptyline and/or nortriptyline, carbamazepine, or gabapentin. Other systemic manifestations of systemic vasculitis require management according to organ involvement and specialty consultation.
Consultations
Systemic vasculitis can involve multiple organ systems. A multispecialty team effort is recommended.
- Neurologist: Neurologist consultation is indicated for diagnosis and treatment of the disease. Neurologist consultation is necessary for electrodiagnostic studies and muscle and nerve biopsy.
- Rheumatologist: Rheumatologist consultation is necessary, especially in systemic vasculitis.
- Physical medicine and rehabilitation specialist: Begin physical therapy and occupational therapy as soon as the patient's condition is stable enough for therapy.
Diet
No special diet is required; however, diet precautions should be provided when corticosteroids are initiated. Patients with diabetes need careful glucose control. Patients should be on potassium supplements if long-term steroid treatment is necessary.
Activity
Most patients in the acute stage of vasculitic neuropathy can be ill if overt signs of other generalized systemic vasculitis are present. Otherwise, activity may be restricted secondary to weakness in the distribution of involved nerves. Encourage ambulation and physical therapy when the patient's condition is stable.
Kernohan JW, Woltman HW. Periarteritis nodosa: a clinicopathologic study with special reference to the nervous system. Arch Neurol Psychiatry. 1938;39:655-86.
Dyck PJ, Benstead TJ, Conn DL, et al. Nonsystemic vasculitic neuropathy. Brain. Aug 1987;110 ( Pt 4):843-53. [Medline].
Davies L, Spies JM, Pollard JD, McLeod JG. Vasculitis confined to peripheral nerves. Brain. Oct 1996;119 ( Pt 5):1441-8. [Medline].
Kissel JT, Riethman JL, Omerza J, Rammohan KW, Mendell JR. Peripheral nerve vasculitis: immune characterization of the vascular lesions. Ann Neurol. Mar 1989;25(3):291-7. [Medline].
Murthy JM, Sundaram C, Meena AK. Nonsystemic vasculitic neuropathy. J Assoc Physicians India. Feb 1998;46(2):204-6. [Medline].
Larrode P, Ramon y Cajal S, Iníguez C, Dominguez M, Sanz JM, Morales F. [Isolated vasculitis of the peripheral nervous system]. Neurologia. May 1997;12(5):197-9. [Medline].
Collins MP, Mendell JR, Periquet MI, Sahenk Z, Amato AA, Gronseth GS. Superficial peroneal nerve/peroneus brevis muscle biopsy in vasculitic neuropathy. Neurology. Sep 12 2000;55(5):636-43. [Medline].
Sanchez GM, Villanueva-Haba VE, Sevilla Mantecon T, Mayordomo Fenandez F, Vilchez Padilla JJ. Nonsystemic vasculitic neuropathy. Neurologia. 2002;17:613-5.
Collins MP, Periquet MI, Mendell JR, Sahenk Z, Nagaraja HN, Kissel JT. Nonsystemic vasculitic neuropathy: insights from a clinical cohort. Neurology. Sep 9 2003;61(5):623-30. [Medline].
Mathew L, Talbot K, Love S, Puvanarajah S, Donaghy M. Treatment of vasculitic peripheral neuropathy: a retrospective analysis of outcome. QJM. Jan 2007;100(1):41-51. [Medline].
Kissel JT, Slivka AP, Warmolts JR, Mendell JR. The clinical spectrum of necrotizing angiopathy of the peripheral nervous system. Ann Neurol. Aug 1985;18(2):251-7. [Medline].
Kararizou E, Davaki P, Karandreas N, Davou R, Vassilopoulos D. Nonsystemic vasculitic neuropathy: a clinicopathological study of 22 cases. J Rheumatol. May 2005;32(5):853-8. [Medline].
Jayne D. Evidence-based treatment of systemic vasculitis. Rheumatology (Oxford). Jun 2000;39(6):585-95. [Medline].
Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. Feb 1994;37(2):187-92. [Medline].
Frohnert PP, Sheps SG. Long-term follow-up study of periarteritis nodosa. Am J Med. Jul 1967;43(1):8-14. [Medline].
Moore PM, Cupps TR. Neurological complications of vasculitis. Ann Neurol. Aug 1983;14(2):155-67. [Medline].
Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). Jan 1999;78(1):26-37. [Medline].
Sehgal M, Swanson JW, DeRemee RA, Colby TV. Neurologic manifestations of Churg-Strauss syndrome. Mayo Clin Proc. Apr 1995;70(4):337-41. [Medline].
Abgrall S, Mouthon L, Cohen P, et al. Localized neurological necrotizing vasculitides. Three cases with isolated mononeuritis multiplex. J Rheumatol. Mar 2001;28(3):631-3. [Medline].
Hawke SH, Davies L, Pamphlett R, et al. Vasculitic neuropathy. A clinical and pathological study. Brain. Oct 1991;114 ( Pt 5):2175-90. [Medline].
Johnson R, Griffin J. Current Therapy in Neurologic Disease. 5th ed. Philadelphia: BC Decker;. 1997.
Kissel JT, Mendell JR. Vasculitic neuropathy. Neurol Clin. Aug 1992;10(3):761-81. [Medline].
Olney RK. Neuropathies associated with connective tissue disease. Semin Neurol. 1998;18(1):63-72. [Medline].
Said G. Vasculitic neuropathy. Curr Opin Neurol. Oct 1999;12(5):627-9. [Medline].
Satoi H, Oka N, Kawasaki T, et al. Mechanisms of tissue injury in vasculitic neuropathies. Neurology. Feb 1998;50(2):492-6. [Medline].
Vedeler CA. Inflammatory neuropathies: update. Curr Opin Neurol. Jun 2000;13(3):305-9. [Medline].

