Management and Outcomes of Cardiac Sarcoidosis: Insights From the Nationwide MYSTICS Registry
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.
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Authors: Chisato Izumi, Yusuke Yoshikawa, Issei Komuro, Ken-ichi Hirata, Koh Ono, Yasushi Sakata, Fumio Terasaki, Takafumi Suda, Hidekazu Tanaka, Toshihiro Tamura, Takeshi Kitai, Takao Kato, Hiroyuki Takahama, Yoichi Takaya, Mika Mori, Yuichi Baba, Kimi Sato, Shunsuke Ishii, Kengo Kusano, Kunihiro Nishimura, Masashi Amano, Kenji Moriuchi, Takahiro Sakamoto, Satoshi Yuda, Yoshihiro Morino, Takahito Nasu, Shingo Sasaki, Yuji Ishida, Nobutaka Nagano, Toshiyuki Yano, Naka Sakamoto, Shota Tokuno, Toru Takahashi, Yoshiya Kato, Hirokazu Komoriyama, Naoya Matsumoto, Yu Horiuchi, Michihiro Yoshimura, Kazuo Ogawa, Tohru Minamino, Yuya Matsue, Kenji Yodogawa, Eisuke Amiya, Shunsuke Kiuchi, Satoshi Hida, Kazuhiro Satomi, Yukio Hiroi, Takashi Nakagawa, Atsushi Suzuki, Yasuo Okumura, Daisuke Kitano, Takashi Ashikaga, Mao Matsuyama, Kyoko Soejima, Shinsuke Takeuchi, Akiko Ueda, Nobuhiro Tanaka, Yoichi Iwasaki, Yuichi Ono, Yoshihiro J. Akashi, Keisuke Kida, Mio Ebato, Miki Tsujiuchi, Yuichiro Iida, Yu Takigami, Yuko Eda, Hidefumi Nakahashi, Tomoo Nagai, Takeshi Ijichi, Michiko Daimon, Shunichi Kushida, Tomoko Ishizu, Takanori Yasu, Takusi Sugiyama, Kenji Harada, Yusuke Ishiyama, Hideo Fujita, Hisataka Maki, Shintaro Nakano, Nogiku Niwamae, Masaru Obokata, Tomonari Harada, Yoshikazu Yazaki, Hiromi Tsuchiya, Shusaku Maruyama, Koji Yoshie, Kazuya Yamamoto, Yuichi Katagiri, Ken Umetani, Keita Sano, Ryuzo Nawada, Yusuke Hattori, Norio Kanamori, Hideki Saito, Toshiaki Suzuki, Yuichiro Maekawa, Yoshihisa Naruse, Masaki Matsunaga, Yoshihiro Seo, Kosuke Nakasuka
Institutions: University of Tsukuba, Kyoto University, The University of Osaka, The University of Tokyo, Tohoku University, Kōchi University, Kindai University Sakai Hospital, Osaka University of Pharmaceutical Sciences, Kindai University, Hamamatsu University School of Medicine, Okayama University, Kitasato University, International University of Health and Welfare, Kobe University, Kanazawa University Hospital, Tenri Hospital, National Cerebral and Cardiovascular Center, Kochi Medical School Hospital, Hyogo Medical University, Kanazawa Hospital