ESR in MAS/HLH

Q: Highly elevated Erythrocyte Sedimentation Rate (ESR) is expected in adults' Still's disease and Macrophage Activation Syndrome (MAS), and is a good biomarker to follow for the severity of the disease.

A) True

B) False

Answer: B

Contrary to what is expected, ESR in both adults' Still's disease and MAS is not a good biomarker. It may be even normal or lower than expected due to low or normal levels of haptoglobin and fibrinogen.

MAS is a subtype of hemophagocytic lymphohistiocytosis (HLH) and may occur in systemic rheumatic disease. It may occur in Still's disease, and is a leading cause of mortality. MAS and HLH are essentially hyperinflammatory states, commonly known as "cytokine storm," and are characterized by fever, hepatosplenomegaly, hyperferritinemia, hypertriglyceridemia, cytopenias, transaminitis, and CNS symptoms.

It is also proposed that MAS/HLH in Still's disease is likely due to infection in the setting of immunosuppression or to reactivation of latent viruses. 

#rheumatology

#immunology

References:

1. Wang R, Li T, Ye S, et al. Macrophage activation syndrome associated with adult-onset Still's disease: a multicenter retrospective analysis. Clin Rheumatol 2020; 39:2379.

2.Bae CB, Jung JY, Kim HA, Suh CH. Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features, predictive factors, and prognosis in 21 patients. Medicine (Baltimore) 2015; 94:e451.

3. Javaux C, El-Jammal T, Neau PA, et al. Detection and Prediction of Macrophage Activation Syndrome in Still's Disease. J Clin Med 2021;11.