Treatment options for refractory Wegener's granulomatosis: a role for rituximab?

Wegener's granulomatosis (WG) is a small-vessel vasculitis of unknown etiology, involving mainly the upper airways, lungs and kidneys. Organ inflammatory damage is mediated by anti-neutrophil cytoplasm antibodies, and their detection is a component of the diagnostic work-up as well as clinical signs and symptoms, and histopathological biopsy abnormalities. Conventional therapeutic regimens, such as cyclophosphamide and corticosteroids, can be used to induce and maintain disease remission. Alternatively, other cytotoxic agents (eg, methotrexate or mycophenolate mofetil), anti-TNFalpha agents (eg, infliximab or etanercept) or anti-lymphocyte antibodies (eg, rituximab) can be used. Rituximab, a mAb which targets CD20+ B-cells, is currently used in the treatment non-Hodgkin's lymphoma and rheumatoid arthritis, and is being investigated for refractory WG therapy and other autoimmune diseases.