Pharmacotherapy of vasculitis.

The systemic vasculitides are characterized by inflammatory lesions in blood vessels. Therapeutic approaches should be based on the aetiology or pathophysiology of disease. Unfortunately, for many of these disorders neither is fully understood and empirical treatment based on clinical presentation and the pattern of organ involvement is used. This approach is effective in improving survival in the most serious forms. We undertook a systematic literature review to assess the evidence for using drug therapies in vasculitis. Glucocorticoids remain essential for many forms of vasculitis; indeed, in giant-cell arteritis, they may be the only therapy necessary. However, additional immunosuppressive agents are required for other forms of vasculitis: methotrexate in Takayasu's arteritis and non-renal small-vessel vasculitis and cyclophosphamide for classic Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome with poor prognostic features. Subsequent disease control is with low-dose glucocorticoid and azathioprine or methotrexate. Biologic therapy is being used in resistant cases. Patients experience significant short- to medium-term toxicity, especially infection and steroid side effects. Late sequelae due to high cumulative doses of cyclophosphamide include infertility and malignancy. Such risks are being reduced due to more judicious use of short courses of cyclophosphamide followed by substitution by safer agents.