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.
-
Diagnostic classification of peripheral vasculitic neuropathy (PVN).
