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Systemic Amyloid Protein a Amyloidosis Involving the Heart in a Patient with Rheumatoid Arthritis

류마티스 관절염 환자에서 심장 조직 검사로 확진된 아밀로이드 단백 A 심장 아밀로이드증

  • Hwang, Ji-won (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Jeon, Eun-Seok (Division of Cardiology, Cardiac and Vascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Ha, Jung Min (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Woo Joo (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kim, Eun (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Yune, Sehyo (Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 황지원 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 전은석 (성균관대학교 의과대학 삼성서울병원 순환기내과 심혈관센터) ;
  • 하정민 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 이우주 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 김은 (성균관대학교 의과대학 삼성서울병원 내과) ;
  • 윤세효 (성균관대학교 의과대학 삼성서울병원 내과)
  • Received : 2013.05.31
  • Accepted : 2013.07.26
  • Published : 2014.06.01

Abstract

Amyloidosis can be identified by the deposition of amyloid fibrils in biopsy specimens from multiple organs, including the heart, kidney, skin, and bowel. Systemic amyloid protein A amyloidosis (AA amyloidosis) is commonly associated with chronic inflammatory diseases or chronic infectious conditions. Cardiac involvement in AA amyloidosis is found in < 1% of reported cases. Here, we report a case of cardiac AA amyloidosis confirmed by heart biopsy in a 54-year-old-female with a medical history of rheumatoid arthritis and stage 4 chronic kidney disease due to renal amyloidosis. She had suffered from progressive aggravation of dyspnea for 2 years. Infiltrative disease involving the heart was suspected by echocardiography, and the patient was diagnosed with AA amyloidosis involving the heart by cardiac biopsy. This is a rare case of cardiac involvement in a patient with systemic AA amyloidosis associated with rheumatoid arthritis.

저자들은 류마티스 관절염으로 치료를 받던 중 신장 조직 검사로 AA 아밀로이드증으로 진단받았던 말기 신부전 환자에서 발생한 심부전 증상과 심초음파 소견을 근거로 심장 아밀로이드증을 의심하였고 심장 조직 검사를 통해 AA 아밀로이드증이 심장 침범하였다는 사실을 면역조직학적으로 확진하였기에 문헌고찰과 함께 보고하는 바이다.

Keywords

References

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