2017 Volume 127 Issue 9 Pages 2105-2110
We report a 35-years-old Japanese female patient suffering from granulomatosis with polyangiitis (GPA). She had orbital granuloma and lung nodules, and she lost her eyesight in the right eye. She was treated for GPA with oral prednisolone and cyclophosphamide. She went into remission; however, she relapsed four months later. We decided to start additional treatment using rituximab, and she went into remission again. After 8 months, her titer of PR3-ANCA rose, and, a month later, we detected CD20 positive B cells, so we started a second course of treatment with rituximab. Although the effectiveness of rituximab for refractory ANCA-associated vasculitis has recently been reported, the details of rituximab maintenance therapy remain unclear.