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

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.

• Authors: N K Loh, M Lucas, S Fernandez, D Prentice
• Journal: Internal medicine journal (Intern Med J) Vol. 42 Issue 12 Pg. 1358-62 (Dec 2012) ISSN: 1445-5994 [Electronic] Australia
• PMID: 23253002 (Publication Type: Case Reports, Journal Article)
• Copyright: © 2012 The Authors; Internal Medicine Journal © 2012 Royal Australasian College of Physicians.

Chemical References

• Antirheumatic Agents
• Interleukin 1 Receptor Antagonist Protein

Topics

• Antirheumatic Agents (administration & dosage, therapeutic use)
• Diagnosis, Differential
• Flow Cytometry
• Humans
• Interleukin 1 Receptor Antagonist Protein (administration & dosage, therapeutic use)
• Killer Cells, Natural
• Lymphocyte Count
• Macrophage Activation Syndrome (complications, diagnosis, drug therapy, immunology)
• Male
• Still's Disease, Adult-Onset (complications, diagnosis, immunology)
• Young Adult