Background and aims In recent years hemophagocytic syndrome (HS) has been increasingly reported in patients with systemic lupus erythematosus (SLE).

Methods We reviewed the medical records of adult patients with SLE and HS for a recent 6 years period (2010–2015). The diagnosis of SLE was made using ACR criteria and of HS using Hunter criteria.

Results Among 110 consecutive patients, 13 (12 women) was identified having HS. The mean age was 37.69+/-11.4 years (21-68). HS revealed lupus in 3 patients. Fever, pericarditis and splenomegaly were found in 100%, 54% and 46% at presentation of HS. Bone marrow aspiration indicated hemophagocytosis in all patients. Laboratory features were bicytopenia or pancytopenia, high C-reactive protein level (mean 93 mg/L) hyperferritinemia (mean 11.082 ng/ml), hypertriglyceridemia (mean 4.2 g/L) in all patients. All patients had anti-nuclear antibodies when the HS occurred. Serum complement C3 was low in 10 patients. HS was associated with a lupus flare in 8 patients. Infections was diagnosed in 11 patients. Both conditions was considered present in 6 patients.

Corticosteroids were initially administered in all patients. Immunosuppressant therapy was used together with corticosteroids in 7 patients. Intravenous immunoglobulin was given in 3 cases. Anti-tuberculosis treatment was used also as first line treatment in 4 patients with life threatening presentation. All patients had a good outcome with a mean follow-up of 25 months.

Conclusions The occurrence of HS was most frequently associated with the SLE disease activity and bacterial infection. Profound cytopenia, high SLEDAI score are the characteristics of SLE patients with HS in our series.