Using anti-SRP antibody, we examined the clinical features of 3 cases of dermatomyositis who visited our hospital. The average age of these 3 patients was 57 years and all the cases were female. All 3 patients had Gottron's sign on their hands as a common skin manifestation. They all suffered from muscle weakness and the myogenic enzymes, creatine kinase and aldolase were elevated. Interstitial lung disease and a malignant tumor were each found in 1 of the 3 cases. One patient was treated solely systemic administration of corticosteroid and the remaining 2 patients received a combination therapy of corticosteroid and an immunosuppressive agent. All their symptoms were resolved, and their conditions came under control.
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.