A 28-year-old male presented with a 3-month history of night sweats,  rigors and flu-like symptoms as well as painful joints experienced for  2 months. No previous medical history of significance was reported. The  patient was pyrexial and had multiple enlarged anterior triangle cervical  lymph nodes and a palpable splenomegaly. A lymph node biopsy showed  reactive follicular hyperplasia without features of lymphoma. Bone  marrow examination was negative for both Mycobacterium tuberculosis and malignancy. Due to clinical deterioration and fulfilment of five out  of eight Histiocyte Society HLH Study Group diagnostic criteria, the  HLH-94 treatment protocol was initiated.
During the work-up for precipitants of HLH, findings that confirmed  previously undiagnosed systemic lupus erythematosus (SLE) were  reported as the trigger of HLH. Induction therapy with etoposide and  dexamethasone was administered for 8 weeks and yielded a positive  response. He developed lupus nephritis 2 months later that was  successfully treated with cyclophosphamide. HLH maintenance therapy  with cyclosporine was not initiated, and he remained well on follow-up.