2026-07-12 | 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.
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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2026-06-29 | A case of adult-onset Still disease complicated by macrophage activation syndrome and toxic epidermal necrolysis.
A 56-year-old woman with 1-year recurrent fever, rash, joint swelling (acute exacerbation) was diagnosed with adult-onset Still disease (AOSD) per 1992 Yamaguchi criteria. She initially improved with methylprednisolone, tocilizumab, methotrexate, and iguratimod but relapsed after discontinuing methylprednisolone. Twenty-four hours after first Re Du Ning injection, she developed progressive rash (extensive polymorphic erythema, bullae, and >30% body surface epidermal detachment), high fever, organ impairment, and coagulopathy and was diagnosed with AOSD complicated by macrophage activation syndrome (2004 haemophagocytic lymphohistiocytosis guidelines), toxic epidermal necrolysis, myocardial injury, hepatic impairment, and disseminated intravascular coagulation. Treatment included dexamethasone, methylprednisolone, plasma exchange, cyclosporine, anakinra, ganciclovir, voriconazole, intravenous immunoglobulin, and supportive care, leading to marked improvement in lesions. This case highlights rare AOSD complications and anakinra's efficacy.
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2026-06-27 | Achievement of intermediate treatment targets for Still's disease under tocilizumab treatment: a post-hoc analysis of the phase III trial.
To evaluate the intermediate treatment targets for Still's disease proposed by EULAR/PReS recently, we conducted this post-hoc analysis of the Phase III trial of tocilizumab and its long-term extension to assess the achievement probability of these targets with tocilizumab. Additionally, we assessed the associations of the intermediate treatment targets with long-term outcomes, including glucocorticoid-free clinically inactive disease (CID) and recurrence. Given the predefined glucocorticoid tapering schedule, we also evaluated the achievement of CID irrespective of glucocorticoid dosage when assessing treatment targets at Months 3 and 6. Twenty-one patients were followed for a median of 39.8 months. The week 4 target was achieved in 57.1%, while 47.6% and 38.1% achieved CID at months 3 and 6, respectively. At the final visit, 57.1% and 28.6% achieved CID and glucocorticoid-free CID, respectively. Achievement of the week 4 target and CID at month 6 was associated with subsequent glucocorticoid-free CID (50% vs. 0%, p = 0.01; 62.5% vs. 7.7%, p = 0.01). Month 6 CID achievers had higher baseline swollen joint counts and lower interferon-γ levels. In conclusion, achievement of intermediate treatment targets was associated with long-term CID, suggesting that these targets may also be useful in treatment with tocilizumab. UMIN000012987, UMIN000018414.
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