SynthesisAnnals of the rheumatic diseases2023
The 2022 EULAR/ACR points to consider at the early stages of diagnosis and management of suspected haemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS).
Synthesis in Annals of the rheumatic diseases, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT06339177 (Hemophagocytic Lymphohistiocytosis), which is not on this map. Cited by 86 papers, 4 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Hemophagocytic Lymphohistiocytosis (HLH) Evaluation and Research of Clinical, ImmUnoLogic and TranscriptomE Study
Who cites it
86 citing papers in PubMed, 4 syntheses or guidelines pooled it, 121 citations in OpenAlex.
- Early intensification versus step-up biologic strategies in systemic juvenile idiopathic arthritis: a Bayesian network meta-analysis of remission and safety outcomes.Pediatric rheumatology online journal · 2026Pooled it
- Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project.Rheumatology (Oxford, England) · 2025Pooled it
- EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease.Annals of the rheumatic diseases · 2024Guideline
- Systemic juvenile idiopathic arthritis and adult-onset Still's disease are the same disease: evidence from systematic reviews and meta-analyses informing the 2023 EULAR/PReS recommendations for the diagnosis and management of Still's disease.Annals of the rheumatic diseases · 2024Pooled it
- Review
- Macrophage activation syndrome in systemic lupus erythematous and Still's disease: distinct phenotypes shaped by the underlying diseases.Rheumatology (Oxford, England) · 2026Article
- Diagnostic performance of HScore for macrophage activation syndrome complicating Still's disease in adults: a multicentre case-control study.Rheumatology (Oxford, England) · 2026Article
- Hyperferritinemia: Pathophysiology, Etiologies, and Diagnostic Approach.European journal of haematology · 2026Review
- Early Differential Diagnosis of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome in Children: A Clinical Prediction Model Based on 106 Patients.Pathogens (Basel, Switzerland) · 2026Article
- Loss of IL10 Signaling Promotes TLR9-Driven Liver Dysfunction by Allowing T Cell Receptor-Mediated T Cell Activation.Research square · 2026Article
- Early management of hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a systematic literature review.EULAR rheumatology open · 2026Review
- Acute hemophagocytic lymphohistiocytosis in a patient treated for stone removal via percutaneous nephrolithotomy: a case report and literature review.International journal of surgery case reports · 2026Article
- Macrophage activation syndrome in systemic lupus erythematosus: risk factors and outcomes.Clinical rheumatology · 2026Article
- Machine learning for predicting macrophage activation syndrome in adult patients with Still's disease.Journal of translational autoimmunity · 2026Article
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- Emapalumab use in malignancy-associated hemophagocytic lymphohistiocytosis in the United States: the REAL-HLH study.Blood advances · 2026Article
- CXCL9 as a novel prognostic marker to identify high-risk adults with hemophagocytic lymphohistiocytosis.Blood · 2026Article
26 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
32 authors at 17 institutions in 6 countries.
Funding
Abstract
objectiveHaemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening systemic hyperinflammatory syndromes that can develop in most inflammatory contexts. They can progress rapidly, and early identification and management are critical for preventing organ failure and mortality. This effort aimed to develop evidence-based and consensus-based points to consider to assist clinicians in optimising decision-making in the
methodsA multinational, multidisciplinary task force of physician experts, including adult and paediatric rheumatologists, haematologist/oncologists, immunologists, infectious disease specialists, intensivists, allied healthcare professionals and patients/parents, formulated relevant research questions and conducted a systematic literature review (SLR). Delphi methodology, informed by SLR results and questionnaires of experts, was used to generate statements aimed at assisting early decision-making and optimising the initial care of patients with HLH/MAS.
resultsThe task force developed 6 overarching statements and 24 specific points to consider relevant to early recognition of HLH/MAS, diagnostic approaches, initial management and monitoring of HLH/MAS. Major themes included the simultaneous need for prompt syndrome recognition, systematic evaluation of underlying contributors, early intervention targeting both hyperinflammation and likely contributors, careful monitoring for progression/complications and expert multidisciplinary assistance.
conclusionThese 2022 EULAR/American College of Rheumatology points to consider provide up-to-date guidance, based on the best available published data and expert opinion. They are meant to help guide the initial evaluation, management and monitoring of patients with HLH/MAS in order to halt disease progression and prevent life-threatening immunopathology.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.