Vasculitic Neuropathy Treatment & Management
- Author: Abbas Mehdi, MD; Chief Editor: Nicholas Lorenzo, MD, MHA, CPE more...
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.
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.
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.
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. 1987 Aug. 110 ( Pt 4):843-53. [Medline].
Davies L, Spies JM, Pollard JD, McLeod JG. Vasculitis confined to peripheral nerves. Brain. 1996 Oct. 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. 1989 Mar. 25(3):291-7. [Medline].
Murthy JM, Sundaram C, Meena AK. Nonsystemic vasculitic neuropathy. J Assoc Physicians India. 1998 Feb. 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. 1997 May. 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. 2000 Sep 12. 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.
Vrancken AF, Said G. Vasculitic neuropathy. Handb Clin Neurol. 2013. 115:463-83. [Medline].
Gwathmey KG, Burns TM, Collins MP, Dyck PJ. Vasculitic neuropathies. Lancet Neurol. 2014 Jan. 13(1):67-82. [Medline].
Collins MP, Periquet MI, Mendell JR, Sahenk Z, Nagaraja HN, Kissel JT. Nonsystemic vasculitic neuropathy: insights from a clinical cohort. Neurology. 2003 Sep 9. 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. 2007 Jan. 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. 1985 Aug. 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. 2005 May. 32(5):853-8. [Medline].
Jayne D. Evidence-based treatment of systemic vasculitis. Rheumatology (Oxford). 2000 Jun. 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. 1994 Feb. 37(2):187-92. [Medline].
Frohnert PP, Sheps SG. Long-term follow-up study of periarteritis nodosa. Am J Med. 1967 Jul. 43(1):8-14. [Medline].
Moore PM, Cupps TR. Neurological complications of vasculitis. Ann Neurol. 1983 Aug. 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). 1999 Jan. 78(1):26-37. [Medline].
Sehgal M, Swanson JW, DeRemee RA, Colby TV. Neurologic manifestations of Churg-Strauss syndrome. Mayo Clin Proc. 1995 Apr. 70(4):337-41. [Medline].
Hirahara T, Yamashita S, Misumi Y, Kawakami K, Hori H, Honda S, et al. Gait disturbance due to foot drop is refractory to treatment in nonsystemic vasculitic neuropathy. Eur Neurol. 2014. 71(3-4):180-6. [Medline].
Abgrall S, Mouthon L, Cohen P, et al. Localized neurological necrotizing vasculitides. Three cases with isolated mononeuritis multiplex. J Rheumatol. 2001 Mar. 28(3):631-3. [Medline].
Hawke SH, Davies L, Pamphlett R, et al. Vasculitic neuropathy. A clinical and pathological study. Brain. 1991 Oct. 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. 1992 Aug. 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. 1999 Oct. 12(5):627-9. [Medline].
Satoi H, Oka N, Kawasaki T, et al. Mechanisms of tissue injury in vasculitic neuropathies. Neurology. 1998 Feb. 50(2):492-6. [Medline].
Vedeler CA. Inflammatory neuropathies: update. Curr Opin Neurol. 2000 Jun. 13(3):305-9. [Medline].