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Intern Med J. 2012 Dec;42(12):1358-62. doi: 10.1111/imj.12002.

Successful treatment of macrophage activation syndrome complicating adult Still disease with anakinra.

Author information

1
Department of Internal Medicine, Royal Perth Hospital, Perth, Western Australia, Australia. nelson.loh@health.wa.gov.au

Abstract

A previously healthy 20-year-old man presented with adult Still disease (ASD). He developed life-threatening macrophage activation syndrome (MAS), which was refractory to standard immunosuppression but responded dramatically to the IL-1 receptor antagonist anakinra. Subsequent immunological investigations included assessment of the perforin expression of natural killer (NK) cells and CD8+ T cells, which confirmed MAS.

PMID:
23253002
DOI:
10.1111/imj.12002
[Indexed for MEDLINE]

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