Myocardial Damage and Microvasculopathy in a Patient With Systemic Sclerosis

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).

Figure.

¹²³I-β-methyl-p-iodophenyl-pentadecanoic acid (BMIPP)/²⁰¹thallium (Tl) dual myocardial single-photon emission computed tomography images demonstrate anteroseptal low perfusion with slight reversed-mismatch with BMIPP (A: Left, BMIPP; Middle: Tl; Right: mismatch). Endomyocardial biopsy shows spotty replacement fibrosis (B; Azan, ×100) with mild cell infiltration (C; H&E, ×400; arrowheads show vessels) and thickened small vessel wall with mild fibrosis under the intima (arrows) (D, H&E; E, Azan; ×400).

BMIPP/Tl SPECT indicated coronary microvessel dysfunction, and the microscopic findings suggested microvessel disease. Thus, microvasculopathy may induce myocardial damage in SSc.¹

Disclosures

K.M. is a member of Circulation Journal’s Editorial Team.

Conflicts of Interest

The authors declare no conflicts of interest.

Reference

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