Macrophage activation syndrome in adult-onset Still disease

When should we suspect macrophage activation syndrome in patients with adult-onset Still disease?

Bhaskar Dasgupta, MD: Macrophage activation syndrome, as I explained in my talk [see Rheumatological emergencies], can be primary or secondary. Still disease is an important secondary cause of macrophage activation syndrome.

In Still disease whenever the patient presents very acutely unwell, we always look for other features of macrophage activation. The features are changes in their cell lines, cytopenias, elevation of triglycerides, and very high levels of ferritin—it goes up in thousands or tens of thousands. We need to recognize that. What we do for diagnosis is a bone marrow [examination], and we look for hemophagocytosis. If it is present in the bone marrow, we use medications such as cyclosporine, which we can use even intravenously for treatment.

Still disease is a very important rheumatological cause of macrophage activation syndrome.