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

• Published in: Internal medicine journal Vol. 42; no. 12; pp. 1358 - 1362
• Main Authors: Loh, N. K., Lucas, M., Fernandez, S., Prentice, D.
• Format: Journal Article
• Language: English
• Published: Australia Blackwell Publishing Ltd 01.12.2012
• Subjects: adult Still's disease; anakinra; Antirheumatic Agents - administration & dosage; Antirheumatic Agents - therapeutic use; Diagnosis, Differential; Flow Cytometry; haemophagocytic lymphohistiocytosis; Humans; Interleukin 1 Receptor Antagonist Protein - administration & dosage; Interleukin 1 Receptor Antagonist Protein - therapeutic use; Killer Cells, Natural; Lymphocyte Count; macrophage activation syndrome; Macrophage Activation Syndrome - complications; Macrophage Activation Syndrome - diagnosis; Macrophage Activation Syndrome - drug therapy; Macrophage Activation Syndrome - immunology; Male; natural killer cell; Still's Disease, Adult-Onset - complications; Still's Disease, Adult-Onset - diagnosis; Still's Disease, Adult-Onset - immunology; Young Adult
• ISSN: 1444-0903; 1445-5994
• DOI: 10.1111/imj.12002

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.