Macrophage Activated Syndrome associated with Adult Onset Still Disease

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is an underrecognized hyperinflammatory condition with a high mortality, characterized by inappropriate survival of histiocytes and cytotoxic T cells (CTLs), leading to a cytokine storm, haemophagocytosis and multi-organ damage (1). The currently accepted terminology includes two forms of HLH: the primary or familial HLH (fHLH) and the secondary HLH (sHLH) (2). The sHLH associated with rheumatic conditions is known as macrophage activation syndrome (MAS). MAS can develop at any time during the evolution of the rheumatic diseases: at the beginning, during flare of the disease or during intercurrent infections – mostly with viral trigger. We present a case of MAS in an patient with adult-onset Still Disease. It is of great importance to be recognized in early stages, because if promptly treated it might respond well to combination therapy of glucocorticoids and IL1- blockers.