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

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Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening systemic hyperinflammatory syndromes. Systemic hyperinflammation and HLH/MAS can occur in almost any inflammatory condition, but there are certain predisposing conditions and inflammatory triggers. These include rheumatic diseases, malignancies, metabolic diseases and genetic immune disorders.

HLH and MAS are characterized by fever, increased systemic and central nervous system inflammation, low blood cell counts, coagulopathy, and hepatitis. It can lead to multiple organ dysfunction, shock and death. Both HLH and MAS can progress very quickly, so early detection and management are important. But identifying at-risk patients is difficult.

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

Result Paper – Published History of arthritis And at the same time Arthritis and Rheumatology – Includes 6 overarching statements and 24 specific points-of-consideration.
Major themes include the need for prompt syndrome recognition and systematic evaluation
In addition to underlying contributors, early interventions that target both hyperinflammation and its potential contributors. They emphasize the need for careful monitoring of progress and complications, as well as the need for specialist multidisciplinary support. A helpful flowchart is provided to outline the key steps in the evaluation, investigation, and treatment of individuals with hyperinflammation and suspected HLH/MAS.

EULAR hopes that these new points-to-consider will aid in the early assessment, management and monitoring of individuals with HLH/MAS to halt disease progression and prevent life-threatening immunopathology.


Journal Reference:

Shakouri, B., etc. (2023). 2022 EULAR/ACR Points to consider early in the diagnosis and management of suspected hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS). History of arthritis.

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