An Unusual Presentation of Systemic Lupus Erythematosus as Hemophagocytic Lymphohistiocytosis in a Male.

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening hyper-inflammatory condition characterized by excessive activation of macrophages and T cells resulting in multi-organ dysfunction. HLH can be primary/familial or secondary to infections, malignancy, immunosuppression, and autoimmune conditions. Systemic lupus erythematosus (SLE) is an autoimmune condition that can predispose to HLH. SLE, as other immune conditions is more common in females than in males. However, the occurrence of SLE in males and subsequent predisposition to HLH is rare. We report the case of a 22-year-old gentleman who presented with fever for three months and one day of altered sensorium prior to admission. On evaluation, he fulfilled five out of the 17 diagnostic criteria for SLE. His bone marrow biopsy showed hemophagocytosis and met five out of the eight diagnostic criteria of HLH, and a diagnosis of HLH secondary to SLE was made. He was treated with pulse doses of intravenous methylprednisolone and azathioprine and showed dramatic improvement. A high index of suspicion is essential for the diagnosis of HLH and prompt initiation of treatment is of utmost importance for tackling such a rapidly progressive life-threatening condition.