New points-to-consider in the diagnosis, treatment of systemic hyperinflammatory syndromes

NewsGuard 100/100 Score

Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening systemic hyperinflammatory syndromes. Systemic hyperinflammation and HLH/MAS can occur in nearly any inflammatory state, but there are certain predisposing conditions and inflammatory triggers. This includes rheumatic diseases, malignancies, metabolic diseases, and genetic immune problems.

HLH and MAS are characterized by fever, increased systemic and central nervous system inflammation, low blood cell count, coagulopathy, and hepatitis. This can lead to multiple organ dysfunction, shock, and death. Both HLH and MAS can progress very quickly, so early identification and management are critical. But it is hard to identify at-risk patients.

To support this, EULAR has developed new evidence- and consensus-based points-to-consider to support clinicians in the diagnosis, treatment, and monitoring of HLH/MAS. The work was completed by an expert task force of adult and pediatric rheumatologists, hematologists, oncologists, immunologists, infectious disease specialists, intensivists, and allied healthcare professionals - as well as patients and their parents.

The resulting paper - published in the Annals of Rheumatic Disease and simultaneously in Arthritis & Rheumatology - includes 6 overarching statements and 24 specific points-to-consider.
Major themes include the need for prompt syndrome recognition and systematic evaluation
of underlying contributors, alongside early intervention that targets both the hyperinflammation and its likely contributors. They also stress the need for careful monitoring of progression and complications, as well as the need for expert multidisciplinary assistance. A helpful flowchart is provided to outline the key stages of assessment, investigation, and treatment in people with hyperinflammation and suspected HLH/MAS.

EULAR hopes these new points-to-consider will help guide the initial evaluation, management and monitoring of people with HLH/MAS in order to halt disease progression and prevent life-threatening immunopathology.

 

Source:
Journal reference:

Shakoory, B., et al. (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). Annals of the Rheumatic Diseases. doi.org/10.1136/ard-2023-224123.

Comments

The opinions expressed here are the views of the writer and do not necessarily reflect the views and opinions of News Medical.
Post a new comment
Post

While we only use edited and approved content for Azthena answers, it may on occasions provide incorrect responses. Please confirm any data provided with the related suppliers or authors. We do not provide medical advice, if you search for medical information you must always consult a medical professional before acting on any information provided.

Your questions, but not your email details will be shared with OpenAI and retained for 30 days in accordance with their privacy principles.

Please do not ask questions that use sensitive or confidential information.

Read the full Terms & Conditions.

You might also like...
Study reveals inflammation's role in long COVID's lingering effects