Abstract

Background

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.

Objective

To reveal the impact of approval of tafamidis and technetium-scintigraphy on the diagnosis of ATTR cardiomyopathy.

Methods

Ten institutes participated in this study on the pathology consultation of amyloidosis and shared rabbit polyclonal anti-κ_116-133, anti-λ_118-134, and anti-transthyretin_115-124 antibodies. Proteomic analysis was performed when the typing diagnosis by immunohistochemistry was unavailable.

Results

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.

Conclusions

The approval of tafamidis and technetium-scintigraphy raised the awareness of ATTR cardiomyopathy, leading to an upsurge in ATTR-positive cardiac biopsy cases.

中文翻译：

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日本转甲状腺素蛋白阳性心脏活检病例数量急剧增加：全国淀粉样变性分型诊断病理会诊获得的证据

摘要

背景

2019年、2020年和2022年，日本政府批准使用tafamidis和两种锝闪烁显像术治疗转甲状腺素蛋白淀粉样蛋白（ATTR）心肌病，并公布了tafamidis治疗的患者标准。2018年，我们启动了全国淀粉样变性病理会诊。

客观的

揭示他法米迪和锝闪烁扫描的批准对 ATTR 心肌病诊断的影响。

方法

十家机构参与了本次淀粉样变性病理会诊研究，并共享兔多克隆抗κ _116-133、抗λ _118-134和抗运甲状腺素蛋白_115-124抗体。当无法通过免疫组织化学进行分型诊断时进行蛋白质组学分析。

结果

在2018年4月至2022年7月收到的5400例咨询病例中，通过免疫组织化学法确定了4420例刚果红阳性病例中的4119例为淀粉样变性。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，1503 例 ATTR 呈阳性。与前 12 个月相比，过去 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。

结论

他法米迪和锝闪烁扫描的批准提高了人们对 ATTR 心肌病的认识，导致 ATTR 阳性心脏活检病例激增。