No association of HLA-DRB1*15:01 and HLA-DRB1*11 in systemic juvenile idiopathic arthritis-associated macrophage activation syndrome and other secondary hemophagocytic lymphohistiocytoses

EULAR Rheumatology Open 2026 Open access

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Authors: Claas H. Hinze, Oliver Wegehaupt, Sabrina Fuehner, Christoph Kessel, Kai Lehmberg, Stephan R. Ehl, D. Foell
DOI: 10.1016/j.ero.2026.01.003
Cited by: 0
References: 8

Abstract

Systemic juvenile idiopathic arthritis (sJIA), often referred to together with adult-onset Still’s disease (SD) under the umbrella term SD, is a prototypic autoinflammatory condition characterised by systemic inflammation and a risk of macrophage activation syndrome [1]. For consistency, we use the term sJIA throughout the manuscript when referring to paediatric patients. In the context of sJIA, the presence of the human leukocyte antigen (HLA)-DRB1*15:01 allele has been strongly linked to severe delayed hypersensitivity-like reactions to interleukin (IL)-1 and IL-6 inhibitors and to the development of lung disease (sJIA-LD) [2].

Fields of Study & Subjects

Autoimmune and Inflammatory Disorders Research Kawasaki Disease and Coronary Complications Inflammasome and immune disorders

Publication details

Journal / source
EULAR Rheumatology Open
Volume
2
Issue
1
Pages
203–205
Publication date
4 February 2026
Language
EN