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Case Report
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Korean J Med. 2014;86(6):739-743. Published online June 1, 2014.
DOI: https://doi.org/10.3904/kjm.2014.86.6.739
- 류마티스 관절염 환자에서 심장 조직 검사로 확진된 아밀로이드 단백 A 심장 아밀로이드증
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황지원1, 전은석2, 하정민1, 이우주1, 김은1, 윤세효1
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1성균관대학교 의과대학 삼성서울병원 내과
2성균관대학교 의과대학 삼성서울병원 순환기내과 심혈관센터
- Systemic Amyloid Protein a Amyloidosis Involving the Heart in a Patient with Rheumatoid Arthritis
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Ji-won Hwang1, Eun-Seok Jeon2, Jung Min Ha1, Woo Joo Lee1, Eun Kim1, Sehyo Yune1
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1Department of Internal Medicine, Sungkyunkwan University School of Medicine, Seoul, Korea
2Division of Cardiology, Cardiac and Vascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
- Corresponding author: Eun-Seok Jeon ,Tel: +82-2-3410-3419, Fax: +82-2-3410-3849, Email: eunseok.jeon@samsung.com
- Received: May 31, 2013; Revised: July 8, 2013 Accepted: July 26, 2013.
- 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.
߽ɾ :전신적 아밀로이드 단백질 A 아밀로이드증(AA 아밀로이드증); AA 아밀로이드 심근증; 울혈성 심부전;
- 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. (Korean J Med 2014;86:739-743)
Keywords :Systemic amyloid protein A amyloidosis (AA amyloidosis), Congestive heart failure, Rheumatic arthritis
