Pediatric macrophage activation syndrome: clinical features and outcomes across diverse etiologies

Clicks: 2
ID: 320014
2026
Free PDF
Article Quality & Performance Metrics
Overall Quality
Not rated
Combines reader engagement with the AI quality analysis. This article has not been analysed, so there is no overall score — reader engagement is measured and shown alongside.
AI Quality Assessment
Not analyzed
Readership in this journal
Emerging

Ranked #173 of 206 articles by views in Lara D. Veeken

Most read Least read

Bar heights use a square-root scale. Only the 120 most-read articles are drawn; the journal has 206 in total.

Mint this article as an NFT
Not yet minted

Create a permanent, verifiable on-chain record of this article on the Scimatic Network. The NFT is held in your Journament account, and you can withdraw it to your own wallet at any time.

5 SUSD one-off · no wallet required
Sign in to mint
Abstract
OBJECTIVES: To characterize clinical features and outcomes of pediatric macrophage activation syndrome (MAS) and examine associations with immune-regulatory genetic variation. METHODS: This retrospective study included 120 children with MAS. Clinical, laboratory, and outcome data were analyzed. A subgroup underwent genetic testing for Hemophagocytic Lymphohistiocytosis (HLH)-related variants. Multivariable logistic regression identified factors associated with outcomes. RESULTS: Etiologies included multisystem inflammatory syndrome in children (MIS-C) (36.7%), systemic juvenile idiopathic arthritis (sJIA) (26.7%), infection-triggered MAS (23.3%), other rheumatologic diseases (10.8%), and lymphoma (2.5%). Intensive care unit (ICU) admission and acute organ failure occurred in 60% and 45%, and mortality was 17.5%. Compared with sJIA, non-sJIA had higher ICU admission (72.7% vs 25.0%, p= 0.02) and mortality (21.6% vs 6.3%, p= 0.04), whereas recurrence was more common in sJIA (28.1% vs 9.1%, p= 0.04). Stratified analyses (MIS-C, other MAS, and sJIA) showed higher ICU admission in MIS-C and other MAS, highest mortality in other MAS, and highest recurrence in sJIA, with rates of 77.3%, 68.2%, and 25.0% (p< 0.001), 6.8%, 36.4%, and 6.3% (p< 0.001), and 2.3%, 15.9%, and 28.1% (p= 0.01), respectively. Among tested patients, 22.4% carried HLH-related variants. Variant positivity was independently associated with higher ferritin, younger MAS onset, recurrence, acute organ dysfunction, and sJIA aetiology. Higher peak ferritin predicted mortality (OR 2.01) and reduced remission off therapy (OR 0.70), whereas sJIA aetiology was associated with lower mortality (OR 0.21). CONCLUSION: Pediatric MAS is etiologically heterogeneous, with inflammatory burden and organ dysfunction driving poor outcomes. Immune-regulatory variants are linked to severe or relapsing disease, supporting phenotype-guided stratification.
Reference Key
openalex_W7167729065 Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Şeyma Türkmen, Hazal Ceren Tuğrul, Murat Hakkı Yarar, Metin Eser, Gürkan Atay, Seher Erdoğan, Betül Sözeri
Journal Lara D. Veeken
Year 2026
DOI
10.1093/rheumatology/keag342
URL
https://doi.org/10.1093/rheumatology/keag342
Keywords Keywords not found

Citations

No citations found. To add a citation, contact the admin at info@scimatic.org

Comments

No comments yet. Be the first to comment on this article.