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- Title
The 2022 EULAR/ACR Points to Consider at the Early Stages of Diagnosis and Management of Suspected Haemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome (HLH/MAS).
- Authors
Shakoory, Bita; Geerlinks, Ashley; Wilejto, Marta; Kernan, Kate; Hines, Melissa; Romano, Micol; Piskin, David; Ravelli, Angelo; Sinha, Rashmi; Aletaha, Daniel; Allen, Carl; Bassiri, Hamid; Behrens, Edward M.; Carcillo, Joseph; Carl, Linda; Chatham, Winn; Cohen, Jeffrey I.; Cron, Randy Q.; Drewniak, Erik; Grom, Alexei A.
- Abstract
Objective: Haemophagocytic 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. Methods: A 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. Results: The 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. Conclusion: These 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.
- Subjects
EUROPE; UNITED States; CONSENSUS (Social sciences); DISEASE progression; HEMOPHAGOCYTIC lymphohistiocytosis; MACROPHAGE activation syndrome; RHEUMATOLOGY; EVIDENCE-based medicine; MEDICAL screening; MEDICAL protocols; RHEUMATOLOGISTS; RESEARCH funding; HEALTH care teams; QUESTIONNAIRES; DECISION making in clinical medicine; PHYSICIANS; EARLY diagnosis; EARLY medical intervention; ONCOLOGISTS; ALLIED health personnel; DELPHI method; PARENTS; DISEASE complications
- Publication
Arthritis & Rheumatology, 2023, Vol 75, Issue 10, p1714
- ISSN
2326-5191
- Publication type
Article
- DOI
10.1002/art.42636