Rheumatic diseases are frequent chronic conditions that impose a great burden to society in terms of losses in quality of life and cumbersome dependency1. Although they are considered benign diseases as a whole, some rheumatic diseases may nevertheless be mortal, especially those characterized by severe inflammation2. Hemophagocytic lymphohistiocytosis (HLH), is a potentially fatal hyperinflammatory syndrome that is characterized by histiocyte proliferation and hemophagocytosis. The most typical presenting signs and symptoms are fever, hepatosplenomegaly, and cytopenias. Less frequently observed clinical findings are neurological symptoms, lymphadenopathy, edema, skin rash, and jaundice3, 4. Common laboratory findings include hypertriglyceridemia, hyperferritinemia, a coagulopathy with hypofibrinogemia, and elevated aminotransferases3, 4. However, HLH should be diagnosed using clinical criteria developed by the Study Group of the Histiocyte Society (Table I) 5, 6. Two forms of the syndrome have been well characterized: a familial HLH and secondary HLH. The diagnosis of familial HLH requires either a positive family history of HLH or the presence of genetic mutations, such as perforin gene mutations7. The pathogenesis is poorly understood. However, the strong link between systemic inflammatory syndrome response and haemophagocytosis coupled with peripheral T-cell expansion