Macrophage activation syndrome in a young patient with polymyositis

Ugeskrift for Læger 2026 Open access

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Authors: Stine Daugaard, Søren Jensen‐Fangel, Mads Nyhuus Bendix Rasch, Morten Aagaard Nielsen
DOI: 10.61409/v04250308
Cited by: 0

Abstract

Haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) is a life-threatening condition characterised by systemic hyperinflammation and is often triggered by infections, malignancies, or autoimmune disease. The HScore is a diagnostic tool used to estimate the likelihood of HLH/MAS based on clinical, biochemical, and histological findings. This case describes a patient with polymyositis developing MAS, indicated by an HScore of 219. He was treated with IV anakinra, steroids, and immunoglobulin, leading to significant improvement. This case emphasises the importance of early MAS recognition and timely intervention in critical conditions.

Fields of Study & Subjects

Autoimmune and Inflammatory Disorders Research Kawasaki Disease and Coronary Complications Inflammatory Myopathies and Dermatomyositis

Publication details

Journal / source
Ugeskrift for Læger
Volume
188
Issue
6
Pages
1–3
Publication date
2 February 2026
Language
DA