Flare-up of eosinophilic granulomatosis with polyangiitis (EGPA ; Churg-Strauss syndrome) in a patient undergoing long-term hemodialysis treatment

  • Sato Aya
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Arimura Yoshihiro
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Shimizu Hideki
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Kubota Sayaka
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Isomura Aya
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Konishi Fumiharu
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Kawashima Soko
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Ikegaya Noriko
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Yoshihara Ken
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Komagata Yoshinori
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Kaname Shinya
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine
  • Ishii Shigeyuki
    Higashiyamato Nangai Clinic
  • Satoh Mamoru
    Ohta General Hospital Foundation, Ohta Nishinouchi Hospital
  • Yamada Akira
    Nephrology & Rhematology, First Department of Internal Medicine, Kyorin University School of Medicine

Bibliographic Information

Other Title
  • 長期維持透析中に再燃した好酸球性多発血管炎性肉芽腫症 (Churg-Strauss症候群) の1例
  • 症例報告 長期維持透析中に再燃した好酸球性多発血管炎性肉芽腫症(Churg-Strauss症候群)の1例
  • ショウレイ ホウコク チョウキ イジ トウセキ チュウ ニ サイネン シタ コウサンキュウセイ タハツ ケッカンエンセイ ニクガシュショウ(Churg-Strauss ショウコウグン)ノ 1レイ

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Description

A 57-year-old woman was admitted to our hospital because of asthma, fever, and purpura. Sixteen years before admission, she had been diagnosed with asthma. Six years before admission, symptoms of EGPA (eosinophilic granulomatosis with polyangiitis) occurred with peripheral neuropathy and rapid progressive glomerulonephritis. Renal biopsy showed necrotizing crescentic glomerulonephritis. Immunosuppressive therapy improved her extra-renal symptoms, and resulted in normalization of her MPO-ANCA titer. However, her renal function did not recover and hemodialysis was initiated. She was treated with maintenance hemodialysis with low-dose prednisolone (PSL) without relapse for 5 years. After reducing the PSL dose from 5 mg per day to 3 mg per day because of infection, her asthma worsened and she exhibited hypereosinophilia, purpura, and fever, with an increased MPO-ANCA titer. On the basis of a diagnosis of EGPA relapse, immunosuppressive therapy, including steroid pulse therapy, was started and led to the remission of vasculitis. Although, it is very rare for patients with EGPA to be treated with maintenance hemodialysis, it is known that ANCA-associated vasculitis can occur during hemodialysis treatment. Physicians should consider the risk of a flare-up of EGPA at the time of asthma exacerbation with marked eosinophilia during maintenance hemodialysis in patients with EGPA, and then should check MPO-ANCA.

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