Successful salvage treatment with baricitinib for macrophage activation syndrome complicating adult-onset Still’s disease during interleukin-6 inhibition: a case report and literature review
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ID: 320773
2026
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Abstract
Adult-onset Still's disease is a systemic autoinflammatory disorder, and macrophage activation syndrome is a life-threatening hyperinflammatory complication. Interleukin-1 and interleukin-6 inhibitors have improved outcomes in refractory adult-onset Still's disease, but optimal management of macrophage activation syndrome, particularly when it develops during biologic therapy, remains uncertain. We report a case of a 49-year-old woman with articular-predominant adult-onset Still's disease who developed fulminant macrophage activation syndrome while receiving high-dose glucocorticoids, tacrolimus and the interleukin-6 receptor inhibitor tocilizumab. At the onset of macrophage activation syndrome, she had persistent fever, cytopenia, hyperferritinemia and liver dysfunction, and bone marrow examination revealed hemophagocytosis. Macrophage activation syndrome persisted despite two courses of intravenous methylprednisolone pulse therapy and continuation of tocilizumab. Tocilizumab was discontinued, and treatment was switched to the oral Janus kinase 1/2 inhibitor baricitinib in combination with glucocorticoids and tacrolimus. After this change, the patient experienced rapid defervescence, marked improvement in blood counts and ferritin levels, and sustained control of articular and systemic disease activity. Glucocorticoids were successfully tapered without relapse, and the patient remained in remission for more than two years without serious infections. To contextualize this case, we reviewed published reports on Janus kinase inhibition in adult-onset Still's disease, including cases complicated by macrophage activation syndrome. Multi-cytokine blockade through Janus kinase 1/2 inhibition, targeting overlapping interleukin-6, interferon-gamma and granulocyte-macrophage colony-stimulating factor pathways, may simultaneously suppress macrophage activation and systemic inflammation. This case highlights the potential of baricitinib as a therapeutic option for refractory adult-onset Still's disease-associated macrophage activation syndrome during interleukin-6 inhibition.
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| Authors | Kazuma Nishisaka, Yo Ueda, I Shirasugi, H. Yamada, T. Okano, Keisuke Nishimura, Sho Sendo, Jun Saegusa |
| Journal | Modern Rheumatology Case Reports |
| Year | 2026 |
| DOI |
10.1093/mrcr/rxag072
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| URL | |
| Keywords | Keywords not found |
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