Translational Autoinflammatory Diseases Section, NIH, Bethesda, Maryland.
Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center and University of Cincinnati, Cincinnati, Ohio, and Hematology/Oncology, University of Western Ontario Schulich School of Medicine & Dentistry, London, Ontario, Canada.
Arthritis Rheumatol. 2023 Oct;75(10):1714-1732. doi: 10.1002/art.42636. Epub 2023 Jul 24.
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.
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.
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.
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.
噬血细胞性淋巴组织细胞增生症（HLH）和巨噬细胞活化综合征（MAS）是危及生命的全身性炎症过度活跃综合征，可在大多数炎症环境中发展。它们可能迅速进展，早期识别和管理对于预防器官衰竭和死亡至关重要。本研究旨在制定基于循证和共识的考虑要点，以帮助临床医生在 HLH/MAS 的早期诊断、治疗和监测阶段优化决策。
一个由医生专家组成的多学科、多国家任务组，包括成人和儿科风湿病学家、血液科医生/肿瘤学家、免疫学家、传染病专家、重症监护专家、辅助医疗保健专业人员以及患者/家长，制定了相关的研究问题并进行了系统文献回顾（SLR）。Delphi 方法学，基于 SLR 结果和专家问卷，用于生成旨在协助早期决策和优化 HLH/MAS 患者初始护理的陈述。
工作组制定了 6 项总体陈述和 24 项具体考虑要点，这些要点与 HLH/MAS 的早期识别、诊断方法、初始管理和监测相关。主要主题包括同时需要迅速识别综合征、系统评估潜在诱因、针对炎症过度活跃和可能诱因的早期干预、仔细监测进展/并发症以及专家多学科协助。
这些 2022 年 EULAR/美国风湿病学会考虑要点提供了基于最佳现有发表数据和专家意见的最新指南。它们旨在帮助指导 HLH/MAS 患者的初始评估、管理和监测，以阻止疾病进展并预防危及生命的免疫病理学。
