ArticleImmunity, inflammation and disease2024
Clinical features of macrophage activation syndrome in adult dermatomyositis: A single-center retrospective case-control study.
Article in Immunity, inflammation and disease, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.
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3 citing papers in PubMed, 3 citations in OpenAlex.
- Characteristics of 21 Patients with Secondary Hemophagocytic Lymphohistiocytosis-Insights from a Single-Center Retrospective Study.Medicina (Kaunas, Lithuania) · 2025Article
- Identification of routine blood derived hematological and lipid indices in ILD through machine learning; a retrospective case-control study.Frontiers in medicine · 2025Article
- Clinical features of macrophage activation syndrome in adult dermatomyositis: A single-center retrospective case-control study.Immunity, inflammation and disease · 2024Article
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8 authors at 1 institution in 1 country.
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Abstract
backgroundLittle is known about the features of macrophage activation syndrome (MAS) in dermatomyositis, especially the association between rapidly progressive interstitial lung disease (RP-ILD) and MAS.
objectiveTo determine the characteristics of MAS in patients with dermatomyositis and their association with RP-ILD.
methodsThis was a retrospective cohort study of 201 dermatomyositis patients at the First Affiliated Hospital of Zhejiang University over a 10-year period.
resultsA total of 22 (10.9%) patients were diagnosed with MAS. The rate of RP-ILD was significantly higher in patients with MAS than in those without MAS (81.8% vs. 17.4%, respectively, p < .001). Multivariate analysis indicated that RP-ILD (p = .019), ferritin level > 1685 ng/mL (p = .007) and hemoglobin < 100 g/L (p = .001) were independent risk factors for MAS. Furthermore, RP-ILD patients with MAS presented more cardiac injury (50.0% vs. 13.3%, respectively, p < .009), central nervous system dysfunction (42.8% vs. 3.4%, respectively, p < .001) and hemorrhage (38.9% vs. 3.3%, respectively, p = .003) than RP-ILD patients without MAS. The 90-day cumulative survival rate for patients with MAS was significantly lower than for those without MAS (18.2% vs. 82.1%, respectively, p < .001).
conclusionMAS was a common and fatal complication of dermatomyositis in our cohort. MAS is closely related to RP-ILD in patients with dermatomyositis. When RP-ILD is present in dermatomyositis patients with abnormal laboratory findings, such as cytopenia and hyperferritinemia, the presence of MAS should be considered.
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