Hemophagocytic Lymphohistiocytosis, A Rare Presentation In Lupus Nephritis

Hemophagocytic lymphohistiocytosis (HLH) is a rare immune disorder that is life threatening if not promptly diagnosed and treated. Its identification, however, remains a diagnostic challenge for clinicians, given the large overlap in presenting symptoms with other conditions, including autoimmune disease and infection. Further adding to the complexity is that HLH encompasses 2 separate forms of disease (Figure1): primary or familial HLH (FHL), and secondary HLH (sHLH), which is often referred to as macrophage activation syndrome (MAS) when associated with rheumatological disease and which can occur in response to robust immunological activation. Open in a separate window Figure 1 Overview of the terminology of hemophagocytic syndromes.