Sato Chikako, Takaya Tomofumi, Mori Shumpei, Hasegawa Kohei, Soga Fumitaka, Tanaka Hidekazu, Watanabe Yoshiaki, Nishii Tatsuya, Kono Atsushi K, Morinaga Yukiko, Ishibashi-Ueda Hatsue, Hirata Ken-Ichi
Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, Japan.
Intern Med. 2017;56(2):163-168. doi: 10.2169/internalmedicine.56.7562. Epub 2017 Jan 15.
Late-onset amyloidogenic transthyretin (ATTR) type familial amyloid polyneuropathy (FAP) shows features distinct from those of early-onset hereditary ATTR type FAP. We herein describe an asymptomatic 68-year-old man with late-onset ATTR type FAP whose serial annual electrocardiograms demonstrated progressive left bundle branch block. Latent but severe cardiac involvement seems to be one feature of late-onset ATTR type FAP, similar to senile systemic amyloidosis (SSA). Early differential diagnosis of late-onset ATTR type FAP from SSA is important because, currently, only the former has new therapeutic options available in Japan. The present case report, therefore, highlights the necessity of careful observation for periodic electrocardiograms.
迟发性淀粉样变甲状腺素运载蛋白（ATTR）型家族性淀粉样多神经病（FAP）表现出与早发性遗传性ATTR型FAP不同的特征。我们在此描述一名68岁无症状的迟发性ATTR型FAP男性患者，其连续年度心电图显示左束支传导阻滞逐渐加重。潜在但严重的心脏受累似乎是迟发性ATTR型FAP的特征之一，类似于老年系统性淀粉样变（SSA）。从SSA中早期鉴别诊断迟发性ATTR型FAP很重要，因为目前在日本只有前者有新的治疗选择。因此，本病例报告强调了定期进行心电图仔细观察的必要性。
