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Antisignal recognition particle-positive polymyositis successfully treated with myeloablative autologous stem cell transplantation
  1. J C Henes1,
  2. F Heinzelmann2,
  3. A Wacker1,
  4. H P Seelig4,
  5. R Klein1,
  6. A Bornemann3,
  7. C Faul1,
  8. L Kanz1,
  9. I Koetter1
  1. 1
    Department of Internal Medicine II (Oncology, Haematology, Immunology, Rheumatology, Pulmology), University Hospital, Tuebingen, Germany
  2. 2
    Department of Radiooncology, University Hospital, Tuebingen, Germany
  3. 3
    Department of Neuropathology, University Hospital, Tuebingen, Germany
  4. 4
    Private Laboratories Seelig, Karlsruhe, Germany
  1. Professor I Koetter, University Hospital, Department of Internal Medicine II, Otfried-Mueller-Strasse 10, D-72076 Tuebingen, Germany; ina.koetter{at}med.uni-tuebingen.de

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Patients with antisignal recognition particle (SRP)-positive myositis usually present with symmetric, progressive muscle weakness and highly elevated levels of serum creatine phosphokinase (CPK).1

A 32-year-old male patient presented with these symptoms (CPK 3601 U/l, normal range 0–190 U/l) in September 2000. Magnetic resonance imaging (MRI) of the thighs revealed a massive oedema (fig 1). After ineffective treatment with glucocorticosteroids, azathioprine, methotrexate, ciclosporine A, mycophenolate mofetil and intravenous immunoglobulins MRI assessed active myositis. The muscle biopsy revealed a necrotising myopathy. Treatment was switched to cyclophosphamide pulses with 750 mg/m2 every 3 weeks and intensified by high-dose cyclophosphamide for stem cell mobilisation with 4000 …

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