Department of Molecular Pathology, University of Fukui, Fukui, Japan.
Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
Amyloid. 2023 Sep;30(3):321-326. doi: 10.1080/13506129.2023.2180334. Epub 2023 Feb 16.
In 2019, 2020 and 2022, the Japanese Government approved the use of tafamidis and two technetium-scintigraphies for transthyretin amyloid (ATTR) cardiomyopathy, and announced the patient criteria for tafamidis therapy. In 2018, we had started a nation-wide pathology consultation of amyloidosis.
To reveal the impact of approval of tafamidis and technetium-scintigraphy on the diagnosis of ATTR cardiomyopathy.
Ten institutes participated in this study on the pathology consultation of amyloidosis and shared rabbit polyclonal anti-κ, anti-λ, and anti-transthyretin antibodies. Proteomic analysis was performed when the typing diagnosis by immunohistochemistry was unavailable.
Out of 5400 consultation cases received from April 2018 to July 2022, the type of amyloidosis by immunohistochemistry was determined in 4119 of the 4420 Congo-red positive cases. The incidences of AA, ALκ, ALλ, ATTR, Aβ2M and others were 3.2, 11.3, 28.3, 54.9, 0.6 and 1.8%, respectively. Out of 2208 cardiac biopsy cases received, 1503 cases were ATTR positive. There were 4.0 and 4.9 times more total cases and ATTR-positive cases, respectively, in the last 12 months as compared to the first 12 months.
The approval of tafamidis and technetium-scintigraphy raised the awareness of ATTR cardiomyopathy, leading to an upsurge in ATTR-positive cardiac biopsy cases.
2019 年、2020 年和 2022 年，日本政府分别批准了塔法米迪和两种锝闪烁显像用于转甲状腺素蛋白淀粉样变（ATTR）心肌病，并公布了塔法米迪治疗的患者标准。2018 年，我们开始了一项全国性的淀粉样变性病理咨询。
揭示塔法米迪和锝闪烁显像获批对 ATTR 心肌病诊断的影响。
10 家机构参与了这项淀粉样变性病理咨询研究，并共享了兔多克隆抗κ、抗λ和抗转甲状腺素蛋白抗体。当免疫组织化学分型诊断不可用时，进行蛋白质组学分析。
2018 年 4 月至 2022 年 7 月期间，我们收到了 5400 例咨询病例，在 4420 例刚果红阳性病例中，通过免疫组化确定了 4119 例淀粉样变性的类型。AA、ALκ、ALλ、ATTR、Aβ2M 等的发生率分别为 3.2%、11.3%、28.3%、54.9%、0.6%和 1.8%。在收到的 2208 例心脏活检病例中，有 1503 例为 ATTR 阳性。与前 12 个月相比，最后 12 个月的总病例数和 ATTR 阳性病例数分别增加了 4.0 倍和 4.9 倍。
塔法米迪和锝闪烁显像的批准提高了对 ATTR 心肌病的认识，导致 ATTR 阳性心脏活检病例激增。
