Fatal case of macrophage activation syndrome (MAS) in a patient with dermatomyositis and cytomegalovirus (CMV) viraemia

Allison Virginia Lange, Salahuddin Kazi, Weina Chen, Arti Barnes

Research output: Contribution to journalArticle


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.

Original languageEnglish (US)
JournalBMJ Case Reports
Publication statusPublished - Jul 30 2018



  • adult intensive care
  • connective tissue disease
  • infectious diseases

ASJC Scopus subject areas

  • Medicine(all)

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