Management and Outcomes of Cardiac Sarcoidosis: Insights From the Nationwide MYSTICS Registry

BACKGROUND: Cardiac sarcoidosis (CS) may lead to serious outcomes, but large-scale data are lacking because of its rarity, diagnostic uncertainty, and heterogeneous clinical presentations. Actual management and outcomes, including recurrence, are not well understood. This study aimed to clarify the actual management and outcomes of CS from a nationwide large-scale registry. METHODS: This multicenter retrospective registry enrolled 2366 consecutive patients with CS. Of these, 1852 patients newly diagnosed between 2012 and 2021 were analyzed. Patients were stratified into 4 groups: definite CS, probable CS, presumed systemic sarcoidosis, and presumed isolated CS based on diagnostic criteria. The primary end point was a composite of all-cause death, heart failure hospitalization, or fatal ventricular arrhythmic event. The secondary end point was recurrence of CS. RESULTS: Cardiac imaging performance rates were high (cardiac magnetic resonance: 64%, 18 F-fluorodeoxyglucose positron emission tomography: 82%), and 974 (53%) patients were diagnosed clinically without histological evidence. During a median 5.1-year follow-up, the primary outcome occurred in 519 (28%, 6.4/100 patient-years) patients, and the 4 groups exhibited different clinical presentations and prognoses; definite CS and presumed isolated CS groups showed a high incidence of the primary end point, fatal ventricular arrhythmic event, hospitalization for heart failure, and cardiovascular death. Most patients (89%) received immunosuppressive therapy, but 291 (18%, 4.1/100 patient-years) patients developed recurrence during steroid tapering, with a 5-year recurrence rate of 17.0%. Recurrence was associated with subsequent primary end point (hazard ratio, 1.38 [95% CI, 1.00–1.89]; P =0.048). CONCLUSIONS: This large-scale multicenter registry showed that clinical presentation and prognosis differ depending on the presence or absence of extracardiac lesions and histological evidence; therefore, it is necessary to recognize the characteristics of each phenotype in managing CS.

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Journal
Circulation Heart Failure
Published
2026-09-10
DOI
https://doi.org/10.1161/circheartfailure.126.014184
Primary Topic
Sarcoidosis and Beryllium Toxicity Research
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article
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article

Management and Outcomes of Cardiac Sarcoidosis: Insights From the Nationwide MYSTICS Registry

Miki Tsujiuchi, Kosuke Nakasuka, Michiko Daimon, Koh Ono et al.
Circulation Heart Failure
Sarcoidosis and Beryllium Toxicity Research
article

Management and Outcomes of Cardiac Sarcoidosis: Insights From the Nationwide MYSTICS Registry

Miki Tsujiuchi, Kosuke Nakasuka, Michiko Daimon, Koh Ono, Kenji Moriuchi, Yoshihiro Morino, Hideo Fujita, Tomoo Nagai, Hirokazu Komoriyama, Shunsuke Kiuchi, Shota Tokuno, Nobutaka Nagano, Shinsuke Takeuchi, Koji Yoshie, Ken Umetani, Mio Ebato, Yukio Hiroi, Daisuke Kitano, Takanori Yasu, Nogiku Niwamae, Takao Kato, Yasushi Sakata, Kunihiro Nishimura, Yuichi Katagiri, Yuichiro Iida, Yuji Ishida, Yu Takigami, Kenji Yodogawa, Takahito Nasu, Issei Komuro, Yoshihiro J. Akashi, Yoshihisa Naruse, Hiromi Tsuchiya, Takafumi Suda, Mika Mori, Kengo Kusano, Yusuke Yoshikawa, Yuichi Baba, Yuko Eda, Hidekazu Tanaka, Fumio Terasaki, Masaki Matsunaga, Takashi Ashikaga, Ryuzo Nawada, Yoichi Takaya, Chisato Izumi, Yusuke Ishiyama, Yuichiro Maekawa, Tomonari Harada, Shusaku Maruyama, Kimi Sato, Takeshi Kitai, Hisataka Maki, Toshihiro Tamura, Shingo Sasaki, Shunsuke Ishii, Kazuya Yamamoto, Kazuo Ogawa, Yoichi Iwasaki, Hiroyuki Takahama, Yoshiya Kato, Yoshikazu Yazaki, Satoshi Hida, Keita Sano, Naka Sakamoto, Eisuke Amiya, Mao Matsuyama, Takusi Sugiyama, Takeshi Ijichi, Naoya Matsumoto, Yuichi Ono, Shunichi Kushida, Masashi Amano, Masaru Obokata, Akiko Ueda, Kazuhiro Satomi, Toshiyuki Yano, Yu Horiuchi, Shintaro Nakano, Toru Takahashi, Satoshi Yuda, Takashi Nakagawa, Tomoko Ishizu, Yusuke Hattori, Hideki Saito, Michihiro Yoshimura, Hidefumi Nakahashi, Tohru Minamino, Kenji Harada, Toshiaki Suzuki, Ken-ichi Hirata, Yoshihiro Seo, Keisuke Kida, Kyoko Soejima, Nobuhiro Tanaka, Yasuo Okumura, Norio Kanamori, Takahiro Sakamoto, Yuya Matsue, Atsushi Suzuki
article en

Abstract

BACKGROUND: Cardiac sarcoidosis (CS) may lead to serious outcomes, but large-scale data are lacking because of its rarity, diagnostic uncertainty, and heterogeneous clinical presentations. Actual management and outcomes, including recurrence, are not well understood. This study aimed to clarify the actual management and outcomes of CS from a nationwide large-scale registry. METHODS: This multicenter retrospective registry enrolled 2366 consecutive patients with CS. Of these, 1852 patients newly diagnosed between 2012 and 2021 were analyzed. Patients were stratified into 4 groups: definite CS, probable CS, presumed systemic sarcoidosis, and presumed isolated CS based on diagnostic criteria. The primary end point was a composite of all-cause death, heart failure hospitalization, or fatal ventricular arrhythmic event. The secondary end point was recurrence of CS. RESULTS: Cardiac imaging performance rates were high (cardiac magnetic resonance: 64%, 18 F-fluorodeoxyglucose positron emission tomography: 82%), and 974 (53%) patients were diagnosed clinically without histological evidence. During a median 5.1-year follow-up, the primary outcome occurred in 519 (28%, 6.4/100 patient-years) patients, and the 4 groups exhibited different clinical presentations and prognoses; definite CS and presumed isolated CS groups showed a high incidence of the primary end point, fatal ventricular arrhythmic event, hospitalization for heart failure, and cardiovascular death. Most patients (89%) received immunosuppressive therapy, but 291 (18%, 4.1/100 patient-years) patients developed recurrence during steroid tapering, with a 5-year recurrence rate of 17.0%. Recurrence was associated with subsequent primary end point (hazard ratio, 1.38 [95% CI, 1.00–1.89]; P =0.048). CONCLUSIONS: This large-scale multicenter registry showed that clinical presentation and prognosis differ depending on the presence or absence of extracardiac lesions and histological evidence; therefore, it is necessary to recognize the characteristics of each phenotype in managing CS.

Circulation Heart Failure
Hyogo Medical University (JP), University of Tsukuba (JP), Okayama University (JP), Tohoku University (JP), Kyoto University (JP), Tenri Hospital (JP), Hamamatsu University School of Medicine (JP), Kochi Medical School Hospital (JP), Kanazawa University Hospital (JP), National Cerebral and Cardiovascular Center (JP), Kindai University Sakai Hospital (JP), Kanazawa Hospital (JP), Kitasato University (JP), Kobe University (JP), The University of Tokyo (JP), Kōchi University (JP), International University of Health and Welfare (JP), Osaka University of Pharmaceutical Sciences (JP), Kindai University (JP), The University of Osaka (JP)
Good health and well-being
Openalex Percentile: Top 11%
Sarcoidosis and Beryllium Toxicity Research
DOIOpen Access PDFOpenAlex
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