M. Bourkia

PS8:156 Hemophagocytic syndrome in systemic lupus erythematosus. a monocentric study of 20 cases

Objectives We reviewed the medical records of adult patients with SLE for a recent 8 years period and identified patients who had developed HS. The diagnosis of SLE was made using ACR criteria (4 or more criteria) and HS has been diagnosing dusing Hunter criteria (5 or more). We conducted statistical analyses to identify the characteristics of those patients in comparison with SLE patients without HS. Results Among 208 consecutive lupus patients, 20 patients (19 women) was identified having HS. The mean age of patients was 35.4+11.4 years (21–68). HS revealed lupus in 7 patients; in the others the delay between diagnosis of SLE and HS was 33 months (1 months – 108). Fever, pericarditis and splenomegaly were found in 95%, 70% and 50% of patients at presentation of HS. Bone marrow aspiration indicated hemophagocytosis in all patients. Cutaneo-mucous and arthritis were present in 95% and 70% of patients at presentation of HS. Bicytopenia or pancytopenia, high C-reactive protein level (mean 74 mg/L) and hyperferritinemia (mean 8687 ng/ml), hypertriglyceridemia (mean 4.35 g/L) were present in all patients. All patients had anti-nuclear and. Anti-double-stranded DNA antibodies were present in all patients. Serum complement C3 was low in 17 patients. HS was associated with a lupus flare in 10 patients. Infections was diagnosed in 11 patients. The most commonly used therapy was corticosteroids, which were initially administered in all patients. Immunosuppressant therapy was used with corticosteroids in 9 patients. Intravenous immunoglobulin was given in 4 cases and Rituximab in one patients. Anti-tuberculosis treatment was the first line treatment in 5 patients. All patients had a good outcome without any mortality with a mean follow-up of 12 months. Compared with SLE patients without HS, those with HS was significantly older and showed more oral ulcerations, a higher serum C-reactive protein level, a higher ferritinemia, higher SLEDAI and H score and, splenomegaly. Conclusion HS was observed in 9,26% Moroccan patients with SLE. Recognition of the cause of HS was particularly challenging because it may mimic the clinical features of the under lying disease or be confused with an infectious complication.

238 Hemophagocytic syndrome in systemic lupus erythematosus : a monocentric review of 13 cases

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.