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Fatal case of macrophage activation syndrome (MAS) in a patient with dermatomyositis and cytomegalovirus (CMV) viraemia
  1. Allison Virginia Lange1,
  2. Salahuddin Kazi1,
  3. Weina Chen2,
  4. Arti Barnes3
  1. 1Internal Medicine, University of Texas Southwestern, Dallas, Texas, USA
  2. 2Pathology, Univ Texas SW Med Ctr Dallas, Dallas, Texas, USA
  3. 3Infectious Diseases, University of Texas Southwestern, Dallas, Texas, USA
  1. Correspondence to Dr Allison Virginia Lange, allison.lange{at}


We describe a case of an adult with dermatomyositis (DM) who presents with a rash, high fevers, tachycardia and hypotension, initially concerning for an infectious aetiology or a DM flare. She was found to have cytomegalovirus viraemia which improved after starting valganciclovir. After extensive workup and lack of improvement with broad-spectrum antimicrobial therapy, intravenous immunoglobulin and steroids, the patient was diagnosed with macrophage activation syndrome after bone marrow biopsy and levels of soluble CD25 (soluble interleukin (IL)-2 receptor) and IL2 were obtained. Unfortunately, despite therapy with dexamethasone, anakinra and etoposide, the patient decompensated and the patient’s family opted for comfort care. The patient subsequently expired in the intensive care unit.

  • infectious diseases
  • adult intensive care
  • connective tissue disease
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  • Contributors AVL was the resident who took care of the patient. She wrote the manuscript of the case report and coordinated all edits between the other authors. AB was the wards attending who took care of the patient. She has read and edited the manuscript significantly and approves this draft. SK was the rheumatology attending who saw the patient as a consultant. He read and edited this draft and approves this version. He also answered numerous questions I had about his decision making when I was writing up the case. WC read the bone marrow biopsy and provided the description of the image. She read the draft and provided significant edits. She approves this manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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