ArticleClinical rheumatology2022
Short-term, low-dose etoposide in refractory adult-onset Still's disease-associated macrophage activation syndrome.
Article in Clinical rheumatology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers, 2 of them syntheses that pooled it.
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Who cites it
16 citing papers in PubMed, 2 syntheses or guidelines pooled it, 22 citations in OpenAlex.
- The Asia-Pacific League of Associations for Rheumatology Consensus Recommendations on the Management of Systemic Juvenile Idiopathic Arthritis (Juvenile Still's Disease).International journal of rheumatic diseases · 2026Guideline
- Assessing the effectiveness of etoposide treatment in adult haemophagocytic lymphohistiocytosis: a systematic review and meta-analysis.Clinical and experimental medicine · 2025Pooled it
- Article
- Macrophage activation syndrome-associated adult onset Still disease treatment: a scoping review of case reports and case series.Proceedings (Baylor University. Medical Center) · 2025Article
- Shock in Adult-Onset Still's Disease Complicated by Macrophage Activation Syndrome: Clinical Characteristics and Prognosis of 14 Patients.International journal of hepatology · 2025Article
- Low-dose emapalumab treatment in refractory macrophage activation syndrome secondary to adult onset still's disease/systemic lupus erythematosus: insights from nine cases.Frontiers in immunology · 2025Article
- Macrophage activation syndrome complicating adult-onset Still's disease: the case for standardized guidelines.Proceedings (Baylor University. Medical Center) · 2025Article
- The Risk Factors of Refractory Adult-Onset Still's Disease.International journal of hepatology · 2025Article
- Efficacy and safety of ruxolitinib in adult patients with refractory rheumatic disease-associated macrophage activation syndrome.Frontiers in immunology · 2025Article
- Macrophage activation syndrome in Sepsis: from pathogenesis to clinical management.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2024Review
- Successful management of infection and macrophage activation syndrome patient using low-dose etoposide: A case report.World journal of radiology · 2024Article
- Management of adult-onset Still's disease: evidence- and consensus-based recommendations by experts.Rheumatology (Oxford, England) · 2024Article
- Unveiling the Mystery of Adult-Onset Still's Disease: A Compelling Case Report.Life (Basel, Switzerland) · 2024Article
- Etoposide Therapy of Cytokine Storm Syndromes.Advances in experimental medicine and biology · 2024Review
- Advances in attractive therapeutic approach for macrophage activation syndrome in COVID-19.Frontiers in immunology · 2023Review
- Rare complications of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis: Time to nip them in the bud.Frontiers in immunology · 2022Review
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
introductionIn this study, we modified the classical regimen of the hemophagocytic lymphohistiocytosis-04 protocol and evaluated the efficacy and safety of short-term, low-dose etoposide in patients with refractory macrophage activation syndrome (MAS) associated with adult-onset Still's disease (AOSD).
methodsA total of 17 patients with refractory AOSD-associated MAS were enrolled and received short-term, low-dose etoposide (100 mg twice a week for four times). Another 11 patients, who were not treated with etoposide, were included as historical controls. Patient information, such as clinical manifestations, laboratory results, treatments, and short-term prognosis, were recorded and analyzed.
resultsIn this case series, 88.24% of the patients with MAS who were treated with short-term, low-dose etoposide had a favorable response in 3 weeks, which was significantly higher (p = 0.017) than that in the patients with MAS who were treated without etoposide (45.45%). The 90-day survival rate after the onset of MAS was significantly higher (p = 0.0029) among the patients in the short-term etoposide group (16/17, 94.12%) than in the control group (5/11, 45.45%).
conclusionThe regimen of short-term (2 weeks), low-dose etoposide was highly effective in the treatment for patients with refractory AOSD-associated MAS with an acceptable safety profile. Key Points • There is no high level evidence to guide the management of refractory MAS-associated AOSD patients. • This study was the first to propose and confirm the efficacy and safety of short-term, low-dose etoposide in the treatment of refractory MAS-associated AOSD patients.
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