ABSTRACT

Standard treatment of systemic vasculitides (SV) that have dissimilar disease presentation and course has been a matter of debate for many years. High daily dose of glucocorticoid (1 mg/kg) concomittant with pulse therapy (500-3000 mg) in severe cases and cyclophosphamide (2 mg/kg/day, per oral or 15 mg/kg/2-4 weekly, intravenous for 3-6 months) has been regarded as standard remission induction regime until recent years. Methotrexate and mycophenolate mofetil have been tried and found to be effective in remission induction in a selected subgroup of patients in the short term follow-up. Azathioprin, methotrexate, mycophenolate mofetil and leflunomide have been successfully used as alternatives to cyclophosphamide in maintenance of remission therapy of SV. However, standard immunosuppressives have failed to achieve in either induction or maintenance of remission in a subgroup of patients with SV. Progressive or persistent disease activity and relapses under standard immunosuppressive treatment for more than six weeks have been defined as resistant vasculitis. Drug resistance or intolerance is another etiology for resistance in a minority at patients. ANCA associated vasculitides (AAV) having the status of most observed and relapsed form of small-medium sized SV have the major place in the treatment of resistant vasculitides. Mycophenolate mofetil has been beneficial in a small group of patients with resistant AAV in the retrospective trials. Single cycle of intravenous immunoglobulin has been shown effective in a short term randomized controlled trial in persistent AAV. Short period of infliximab treatment have been to be successful in patients with resistant AAV in the open studies. Deoxyspergualin and anti-thymosit globulin have an effect for inducing remission in resistant AAV, but with the significant high rates of infection. Rituximab has been found to be promising in small open studies.