Abstract
Wegener’s granulomatosis is a rapidly progressing small vessel vasculitis involving mainly the lungs and kidneys and has antineutrophil cytoplasm antibodies against protease 3 as a common immunological marker. Therapy is aimed at prolonging survival, inducing remission of the active disease and preventiong its relapse. Several immunosuppressive agents are currently used in conventional regimen, and cyclophosphamide is the most commonly used. In patients refractory to such therapies, other drugs have to be used and rituximab (an antibody against CD20+ cells) could be a potentially effective treatment for this subset. The data on its clinical efficacy are only preliminary and further studies are needed.