Steep increase in the number of transthyretin-positive cardiac biopsy cases in Japan: evidence obtained by the nation-wide pathology consultation for the typing diagnosis of amyloidosis

Hironobu Naiki, Aina Yamaguchi, Yoshiki Sekijima, Mitsuharu Ueda, Kenichi Ohashi, Kinta Hatakeyama, Yoshihiko Ikeda, Yoshinobu Hoshii, Yukako Shintani-Domoto, Aya Miyagawa-Hayashino, Hanako Tsujikawa, Jin Endo, Tomio Arai, Yukio Ando

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)

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-κ116-133, anti-λ118-134, and anti-transthyretin115-124 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.

Original languageEnglish
Pages (from-to)321-326
Number of pages6
JournalAmyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis
Volume30
Issue number3
DOIs
Publication statusPublished - 2023

Keywords

  • Cardiac biopsy
  • immunohistochemistry
  • tafamidis
  • technetium-scintigraphy
  • transthyretin amyloid cardiomyopathy

ASJC Scopus subject areas

  • Internal Medicine

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