TY - JOUR
T1 - Fatal case of macrophage activation syndrome (MAS) in a patient with dermatomyositis and cytomegalovirus (CMV) viraemia
AU - Lange, Allison Virginia
AU - Kazi, Salahuddin
AU - Chen, Weina
AU - Barnes, Arti
N1 - Publisher Copyright:
© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.
PY - 2018/7/30
Y1 - 2018/7/30
N2 - 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.
AB - 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.
KW - adult intensive care
KW - connective tissue disease
KW - infectious diseases
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U2 - 10.1136/bcr-2018-225231
DO - 10.1136/bcr-2018-225231
M3 - Article
C2 - 30061130
AN - SCOPUS:85056945900
SN - 1757-790X
VL - 2018
JO - BMJ Case Reports
JF - BMJ Case Reports
ER -