A case of severe dysdialysis due to heart failure complicated with immunoglobulin lambda‒related systemic amyloidosis

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  • Heryon Lee
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Fukuoka Kazuhito
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Kunitomo Rie
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Shimoda Sachiko
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Hyodou Chinatsu
    Setagaya Riumachi Kougenbyou Clinic
  • Kukimoto Hikaru
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Kobayashi Satoshi
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Sato Yuriko
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Takemori Ai
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Miyamoto Ayako
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Ozawa Yuko
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Isomura Aya
    Department of Pathology, Kyorin University School of Medicine
  • Kawakami Takahisa
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Komagata Yoshinori
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine
  • Kaname Shinya
    Department of Nephrology and Rheumatology, Kyorin University School of Medicine

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  • 透析困難症の原因として心アミロイドーシス合併が考えられた1例
  • トウセキ コンナンショウ ノ ゲンイン ト シテ シン アミロイドーシス ガッペイ ガ カンガエラレタ 1レイ

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Abstract

<p>A 75‒year‒old male with a 20‒year history of type I diabetes mellitus was treated for diabetic nephropathy with anti‒glutamic acid decarboxylase antibody positivity, hypertension, diabetic retinopathy, massive proteinuria, and peripheral neuropathy. Later, his renal dysfunction deteriorated； therefore, hemodialysis was started. While he was admitted to hospital for hemodialysis, a rash on his trunk and serum IgA‒λ positivity were observed, and the possibility of systemic amyloidosis was considered, but a gastric endoscopic biopsy showed no amyloid deposition, and echocardiography showed normal function. He started to receive outpatient dialysis at his local clinic, but his blood pressure frequently dropped during the dialysis sessions, and he was often admitted to our hospital due to heart failure. During his third hospitalization, low cardiac function without coronary artery lesions was observed, and the patient suddenly died of ventricular fibrillation. A pathological autopsy was performed to elucidate the cause of death, and findings suggestive of amyloidosis were observed, but immunostaining failed to identify any amyloid precursors. Mass spectrometry led to a diagnosis of amyloid light‒chain amyloidosis with IgA‒λ as the precursor. We experienced a case of systemic amyloidosis with rapid progression of cardiac dysfunction after the initiation of dialysis.</p>

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