Rituximab

Abstract

　Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is known as a systemic vasculitis with unknown etiology whereas ANCA, a hallmark of AAV, is considered to promote pathologic process of these disorders. AAV are classified as microscopic polyangiitis (MPA), granulomatous polyangiitis (GPA), eosinophilic granulomatous polyangiitis (EGPA) and renal limited vasculitis (RLV). There have been some differences of clinical characteristic of AAV between Japan and foreign countries since Japanese patients with AAV contain the higher incidence of MPA, myeloperoxidase (MPO)-ANCA, elderly onset of age and interstitial lung disease (ILD). Regrading to therapy of AAV, rituximab (RTX), a chimeric anti-CD20 monoclonal antibody, targeted to clearance of B cells, have been reported to be effective in AAV patients that are mostly evidenced in foreign countries with GPA patients, launched in USA at 2011 toward GPA and MPA. These clinical applications are also launched in Japan at 2013. As compared with foreign countries, evidences of clinical investigation of RTX in AAV patients is still limited in Japan. We are going to discuss the established evidences of RTX toward treatment of AAV and positioning of RTX among Japanese guideline toward clinical application of AAV 2017.