Myocardial Damage and Microvasculopathy in a Patient With Systemic Sclerosis.

1. Manetti M, Romano E, Rosa I, Guiducci S, Bellando-Randone S, De Paulis A, et al. Endothelial-to-mesenchymal transition contributes to endothelial dysfunction and dermal fibrosis in systemic sclerosis. Ann Rheum Dis 2017; 76: 924 – 934. A 78-year-old Japanese woman with systemic sclerosis (SSc) consulted a rheumatologist because echocardiographic examination had revealed pericardial effusion with normal systolic and diastolic function. Her laboratory data were high-sensitivity troponin T 0.249 ng/mL, C-reactive protein, 0.28 mg/dL, and N-terminal-pro B-type natriuretic peptide, 888.9 pg/mL. ECG findings were unremarkable. Cardiac magnetic resonance imaging showed no T2-weighted high intensity and no late gadolinium enhancement. 123I-β-methyl iodophenyl pentadecanoic acid (BMIPP) and 201thallium (Tl) dual myocardial single-photon emission computed tomography (SPECT) demonstrated anteroseptal low perfusion with slight reversed-mismatch with BMIPP (Figure A). Because coronary angiography showed normal coronary arteries, right ventricular endomyocardial biopsy was performed. Microscopically, there was spotty replacement fibrosis with mild cell infiltration (Figure B,C) and mildly thickened intramural small vessel with mild fibrosis under the intima (Figure D,E). BMIPP/Tl SPECT indicated coronary microvessel Received November 3, 2020; revised manuscript received November 9, 2020; accepted November 11, 2020; J-STAGE Advance Publication released online December 19, 2020 Time for primary review: 5 days Department of Cardiovascular Medicine (H.K., K.M.), Department of Immunology and Rheumatology (M.U.), Department of Radioisotope, Radiology (E.S.), Nagasaki University Graduate School of Biomedical Sciences, Nagasaki; Department of Radioisotope Medicine, Atomic Bomb Disease Institute, Nagasaki University, Nagasaki (T.K.); and Department of Pathology, Nagasaki University Hospital, Nagasaki (M.F.), Japan Mailing address: Hiroaki Kawano, MD, FJCS, Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, 1-7-1 Sakamoto, Nagasaki 852-8501, Japan. E-mail: hkawano@nagasaki-u.ac.jp All rights are reserved to the Japanese Circulation Society. For permissions, please e-mail: cj@j-circ.or.jp ISSN-1346-9843 Myocardial Damage and Microvasculopathy in a Patient With Systemic Sclerosis