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Autologous stem cell transplantation leads to a change in proinflammatory plasma cytokine profile of patients with juvenile dermatomyositis correlating with disease activity
  1. F Bellutti Enders1,2,
  2. E M Delemarre1,
  3. J Kuemmerle-Deschner3,
  4. P van der Torre1,
  5. N M Wulffraat1,
  6. B P Prakken1,
  7. A van Royen-Kerkhof1,#,
  8. F van Wijk1
  1. 1Laboratory of Translational Immunology, Department of Pediatric Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands
  2. 2Division of Allergology, Immunology and Rheumatology, Department of Pediatrics, University Hospital, Lausanne, Switzerland
  3. 3Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany
  1. Correspondence to Dr A van Royen-Kerkhof, Laboratory of Translational Immunology, Department of Pediatric Immunology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, Utrecht, Room KC 03.063.0, Lundlaan 6, Utrecht 3584 EA, The Netherlands; a.vanroyen{at}

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Juvenile dermatomyositis (JDM) is a rare autoimmune disorder, affecting mainly muscles and skin. The mainstay of treatment is high dose corticosteroids, combined with other immunosuppressive drugs.1 In about 30% of patients, the disease cannot be controlled despite multiple treatment interventions. Autologous stem cell transplantation (aSCT) has been reported as a last resort treatment in refractory patients with autoimmune diseases.2 The main hypothesis for the underlying immunological mechanism is that aSCT resets the immune system and restores immune tolerance following profound lymphodepletion and immune suppression.3

Here, we report three patients with refractory JDM that received aSCT (Clinical information, table 1). A short-term follow-up with detailed clinical information (including imaging before and after aSCT, and immune reconstitution after aSCT) of two patients describing complete remission was reported previously.4 The current follow-up of these patients is more than 5 years showing sustained remission. A third patient (#3) has a follow-up of nearly 3 years. Indication of aSCT for patient 3 was refractory muscle and skin inflammation comparable to the other two patients. Whole body MRI prior to aSCT confirmed active myositis. As muscle tests improved substantially after aSCT, MRI was not repeated post aSCT in this patient. Immune reconstitution …

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