• Reumatología clinica · Sep 2014

    Hemophagocytic syndrome as the initial manifestation of systemic lupus erythematosus.

    • César Antonio Egües Dubuc, Miren Uriarte Ecenarro, Carlos Meneses Villalba, Vicente Aldasoro Cáceres, Iñaki Hernando Rubio, and Joaquín Belzunegui Otano.
    • Servicio de Reumatología, Hospital Universitario Donostia, San Sebastián, España. Electronic address: tonoeguesdubuc@hotmail.com.
    • Reumatol Clin. 2014 Sep 1;10(5):321-4.

    IntroductionHemophagocytic 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 ObservationsBoth patients had prolonged fever not associated to an infectious process and did not respond to broad-spectrum antibiotics.DiscussionThe 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.ConclusionsHS 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.Copyright © 2013 Elsevier España, S.L. All rights reserved.

      Pubmed     Free full text   Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…