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Klinefelter's syndrome with lupus encephalitis and retroperitoneal teratoma.

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  • المؤلفون: Wq CN;Wq CN; Eide SE; Eide SE; J H; J H; Ym K; Ym K
  • المصدر:
    Lupus [Lupus] 2018 Aug; Vol. 27 (9), pp. 1559-1561. Date of Electronic Publication: 2018 May 14.
  • نوع النشر :
    Case Reports; Journal Article
  • اللغة:
    English
  • معلومة اضافية
    • المصدر:
      Publisher: SAGE Publications Country of Publication: England NLM ID: 9204265 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1477-0962 (Electronic) Linking ISSN: 09612033 NLM ISO Abbreviation: Lupus Subsets: MEDLINE
    • بيانات النشر:
      Publication: London : SAGE Publications
      Original Publication: Houndmills, Basingstoke, Hampshire, UK : Scientific & Medical Division, Macmillan Press Ltd., c1991-
    • الموضوع:
      Encephalitis/*etiology ; Klinefelter Syndrome/*complications ; Lupus Erythematosus, Systemic/*complications ; Retroperitoneal Neoplasms/*complications ; Teratoma/*complications; Encephalitis/diagnostic imaging ; Humans ; Male ; Middle Aged ; Retroperitoneal Neoplasms/diagnostic imaging ; Teratoma/diagnostic imaging
    • نبذة مختصرة :
      We report a case of a middle-aged male who presented with pyrexia of unknown origin, oral ulcers, rash and bicytopenia. His past medical history included unexplained proteinuria, infertility and joint pain. Initial workup showed a large retroperitoneal soft tissue mass, which has appeared stable for 10 years. He subsequently developed neuropsychiatric symptoms with imaging findings of meningoencephalitis. Concurrent investigations showed elevated ANA, anti-double-stranded DNA (dsDNA) and ESR levels, as well as low complement levels. Bone marrow aspirate samples revealed karyotype of chromosome 47, XXY. The unifying diagnosis was therefore Klinefelter's syndrome (KS) with retroperitoneal teratoma and systemic lupus erythematosus (SLE), with manifestations of cytopenias, myositis, serositis, cutaneous vasculitis, encephalitis and nephritis. Upon commencing immunosuppressive therapy, there was dramatic improvement in his mental state and resolution of imaging findings. This case illustrates that males with KS may develop severe SLE manifestations; hence, increased diagnostic vigilance of KS in males with SLE is important in order to recognize effects of hypogonadism and other associations with KS such as retroperitoneal teratoma.
    • Contributed Indexing:
      Keywords: Klinefelter's syndrome; lupus encephalitis; neuropsychiatric lupus; systemic lupus erythematosus; teratoma
    • الموضوع:
      Date Created: 20180516 Date Completed: 20181211 Latest Revision: 20181211
    • الموضوع:
      20240829
    • الرقم المعرف:
      10.1177/0961203318776107
    • الرقم المعرف:
      29759049