A female patient with systemic lupus erythematosus who developed Goodpasture's syndrome

  • Nakamura Yuya
    Department of Nephrology, Tokyo Metolopolitan Komagome General Hospital
  • Nakagawa Jun
    Department of Nephrology, Tokyo Metolopolitan Komagome General Hospital
  • Shibuya Asuka
    Department of Nephrology, Tokyo Metolopolitan Komagome General Hospital
  • Suzuki Hitoe
    Department of Nephrology, Tokyo Metolopolitan Komagome General Hospital
  • Ando Minoru
    Department of Nephrology, Tokyo Metolopolitan Komagome General Hospital

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Other Title
  • 全身性エリテマトーデス加療中にGoodpasture症候群を発症し血液浄化療法を併用し救命しえた1例
  • 症例報告 全身性エリテマトーデス加療中にGoodpasture症候群を発症し血液浄化療法を併用し救命しえた1例
  • ショウレイ ホウコク ゼンシンセイ エリテマトーデス カリョウチュウ ニ Goodpasture ショウコウグン オ ハッショウシ ケツエキ ジョウカ リョウホウ オ ヘイヨウ シ キュウメイシエタ 1レイ

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Abstract

A 64-year-old female with systemic lupus erythematosus (SLE) was admitted to our hospital. Disease activity had been controlled by oral administration of prednisolone (9mg/day) for recent years. In April 2005, she developed a high fever that did not respond to standard therapy with antipyretics and antibiotics. Therefore, she consulted our hospital and was admitted. After hospitalization, renal function rapidly failed and she finally became oliguric on the 4th day. She was considered to have developed rapidly progressive glomerulonephritis (RPGN) and a combination of hemodialysis (HD), steroid pulse therapy and cyclophophosphamide administration was started. Since serological data on the 9th day demonstrated that serum anti-glomerular basement membrane (GBM) antibody titer was elevated to 300EU, plasma exchange therapy was added. On the 25th day, she suddenly developed pulmonary bleeding followed by acute respiratory failure requiring artificial ventilation. At this point, her disease was clinically diagnosed as Goodpasture's syndrome. Combined therapy consisting of plasma exchange, HD, and steroid pulse was continued and the patient made a good recovery, except for kidney function. She is now doing well on maintenance HD therapy. This is a rare patient who developed Goodpasture's syndrome during the stable course of SLE.

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