title: Hemophagocytic syndrome as the initial manifestation of systemic lupus erythematosus.
abstract: BACKGROUND
  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.
  
  
  METHODS
  Both patients had prolonged fever not associated to an infectious process and did not respond to broad-spectrum antibiotics.
  
  
  CONCLUSIONS
  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.
authors: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España. Electronic address: tonoeguesdubuc@hotmail.com.
authors: César Antonio
authors: CA
authors: Egües Dubuc
authors: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors: Miren
authors: M
authors: Uriarte Ecenarro
authors: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors: Carlos
authors: C
authors: Meneses Villalba
authors: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors: Vicente
authors: V
authors: Aldasoro Cáceres
authors: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors: Iñaki
authors: I
authors: Hernando Rubio
authors: Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España.
authors: Joaquín
authors: J
authors: Belzunegui Otano
mesh_terms: D000293:Adolescent; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis*; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology*; D008297:Male; D008875:Middle Aged
keywords: Anakinra; Hemophagocytic lymphohistiocytosis ;Anakinra; Hemophagocytic syndrome; Linfohistiocitocis hemofagocítica; Lupus eritematoso sistémico; Macrophage activation syndrome; Systemic lupus erythematosus; Síndrome de activación macrofágica; Síndrome hemofagocítico