Hemophagocytic syndrome as the initial manifestation of systemic lupus erythematosus.

نویسندگان

  • César Antonio Egües Dubuc
  • Miren Uriarte Ecenarro
  • Carlos Meneses Villalba
  • Vicente Aldasoro Cáceres
  • Iñaki Hernando Rubio
  • Joaquín Belzunegui Otano
چکیده

INTRODUCTION Hemophagocytic syndrome (HS) occurs in autoimmune diseases and belongs to the hemophagocytic lymphohistiocytosis group of diseases. This paper describes the features of 2 patients with systemic lupus erythematosus (SLE) who presented HS as the initial clinical manifestation. CLINICAL OBSERVATIONS Both patients had prolonged fever not associated to an infectious process and did not respond to broad-spectrum antibiotics. DISCUSSION The diagnosis of HS secondary to SLE is complicated, because it has some features in common, but HS is characterized by hyperferritinemia, hipofibrinogemia, hypertriglyceridemia and a decrease in the erythrocyte sedimentation rate, unlike SLE. HS treatment when associated to SLE is not well established, but steroids and/or immunoglobulins are effective as the initial treatment, and in refractory cases, cyclosporine or cyclophosphamide may be associated. CONCLUSIONS HS can be the initial manifestation of SLE and should be suspected in patients with organ enlargement, cytopenias, clotting disorders, liver disorders and prolonged fever unresponsive to antibiotics. Anakinra may be a treatment option in adult HS associated to SLE.

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عنوان ژورنال:
  • Reumatologia clinica

دوره 10 5  شماره 

صفحات  -

تاریخ انتشار 2014