SynthesisArthritis & rheumatology (Hoboken, N.J.)2023
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 Arthritis & rheumatology (Hoboken, N.J.), 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
What it found
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Who cites it
40 citing papers in PubMed, 39 citations in OpenAlex.
- Invasive aspergillosis in autoimmune inflammatory rheumatic diseases: epidemiology, risk factors, diagnosis, management and challenges.Annals of medicine · 2026Review
- Rapid response to high-dose Anakinra in refractory SLE-associated Haemophagocytic Lymphohistiocytosis: a case report.Oxford medical case reports · 2026Article
- Diagnostic performance of HScore for macrophage activation syndrome complicating Still's disease in adults: a multicentre case-control study.Rheumatology (Oxford, England) · 2026Article
- Beyond the Storm: CXCL9 and the New Era of Precision Hemophagocytic Lymphohistiocytosis Diagnostics.Diagnostics (Basel, Switzerland) · 2026Review
- The Unfolding Mystery of Hemophagocytic Lymphohistiocytosis in Pregnancy: A Case Report.Cureus · 2026Article
- Article
- Guideline for the Diagnosis and Management of Heritable IFNAR1 Deficiency in Oceania.Journal of paediatrics and child health · 2026Review
- Early management of hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a systematic literature review.EULAR rheumatology open · 2026Review
- Development and validation of an early risk-stratification model for hemophagocytic lymphohistiocytosis in severe fever with thrombocytopenia syndrome.PLoS neglected tropical diseases · 2026Article
- CXCL9 as a novel prognostic marker to identify high-risk adults with hemophagocytic lymphohistiocytosis.Blood · 2026Article
- Serum Cytokine Profiling Differentiates Underlying Diseases in Cytokine Storm Syndrome.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
- Case Report: Definite DRESS following phenobarbital exposure with secondary HLH and DAT-positive hemolysis during high-dose IVIG treatment in a child.Frontiers in immunology · 2026Article
- Hyperinflammatory Syndrome of Unknown Origin Complicated by Shock, DIC, Intracranial Hemorrhage and Spontaneous Splenic Rupture: A Case Report.Clinical medicine insights. Case reports · 2026Article
- Secondary hemophagocytic lymphohistiocytosis in pediatric patients: a single-center experience.Frontiers in pediatrics · 2026Article
- Early Risk Factors and Clinical Characteristics of Pediatric Hemophagocytic Lymphohistiocytosis: A Single-Center Retrospective Study.Journal of inflammation research · 2026Article
- Development of a predictive tool for hemophagocytic lymphohistiocytosis/macrophage activation syndrome risk in adult-onset Still's disease.Frontiers in immunology · 2026Article
- Immune-Mediated Necrotizing Myopathy: A Systematic Review of Antibody-Specific Mechanisms and Treatment Outcomes.Cureus · 2025Review
- Article
- Progressive Disseminated Histoplasmosis with Dermatomyositis and Macrophage Activation Syndrome/Hemophagocytic Lymphohistiocytosis.The American journal of tropical medicine and hygiene · 2025Article
- Assessment of Hemophagocytic Lympho-Histiocytosis (HLH) in the Setting of Adult Acute Liver Failure.Journal of viral hepatitis · 2025Article
Corrections and comments
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Authors and funding
31 authors at 16 institutions in 7 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 early stages of diagnosis, treatment and monitoring of HLH/MAS.
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.
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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.