Ha Sang Jin, Kim Woo-Shik, Hwang Seung-Joon, Woo Jong Shin, Shon Il Seok, Bae Jong-Hoa, Kim Kwon Sam
Division of Cardiology, Department of Internal Medicine, School of Medicine, Kyung Hee University, Seoul, Korea.
J Am Soc Echocardiogr. 2009 May;22(5):542.e5-7. doi: 10.1016/j.echo.2009.01.022. Epub 2009 Mar 9.
Secondary (amyloid A [AA]) amyloidosis is a systemic disease characterized by amyloid deposition in many organs, leading to impaired function. Although cardiac involvement may occur with AA amyloidosis, significant deposition of amyloid in the heart is considered an infrequent observation and is rarely the cause of death. It occurs in 5% of patients with poorly controlled chronic inflammatory disease, mainly rheumatoid arthritis, ankylosing spondylitis, and familial Mediterranean fever. The authors report a case of AA amyloidosis diagnosed by rectal and skin biopsies, with cardiac involvement demonstrated by typical echocardiographic features in the presence of low voltage on electrocardiography.
继发性（淀粉样蛋白A[AA]）淀粉样变性是一种全身性疾病，其特征是淀粉样蛋白在许多器官中沉积，导致功能受损。虽然AA淀粉样变性可能累及心脏，但心脏中淀粉样蛋白的大量沉积被认为是罕见的观察结果，很少是死亡原因。它发生在5%控制不佳的慢性炎症性疾病患者中，主要是类风湿性关节炎、强直性脊柱炎和家族性地中海热。作者报告了一例通过直肠和皮肤活检诊断为AA淀粉样变性的病例，心电图显示低电压，典型超声心动图特征证实心脏受累。
