Aggressive autoimmune diseases are often treated by intensive immunosuppressive treatment such as high-dose methylprednisolone and intravenous cyclophosphamide. Autologous hematopoietic stem cell transplantation can facilitate high-dose immunosuppressive therapy (HDIT), which is myeloablative. We describe a 54-year-old female patient with rapidly progressive and refractory interstitial pneumonia due to dermatomyositis, which was successfully treated with high-dose cyclophosphamide and autologous blood stem cell transplantation. Following transplantation, dyspnea disappeared, and arterial blood gas analysis and respiratory function test showed marked improvement. This improvement was confirmed by diminished interstitial shadows on chest X-ray and computed tomography scans. Eighteen months after transplantation, the patient is doing well without symptoms and signs of interstitial pneumonia.
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