Diversity of myocardial involvement in systemic sclerosis: An 8-year study of 95 Japanese patients

Background Myocardial fibrosis has been reported to be an important complication of systemic sclerosis (SSc). Left ventricular (LV) hypertrophy has also been reported, but its significance is unclear. Correlations between cardiac complications and clinical or serologic subsets of SSc have not been previously evaluated by long-term observational studies. Methods We correlated the findings of echocardiography, electrocardiography, and autopsy with clinical and serologic features in 95 patients with SSc who were monitored for vp to 8 years. Results Septal thickness determined by echocardiography was significantly greater in patients with anti-nuclear ribonucleoprotein (nRNP) antibodies than in patients with anti-Scl70 or anticentromere antibodies. Fractional shortening was reduced in patients with diffuse cutaneous involvement or anti-Scl70 antibodies. Ten patients had cor pulmonale, which was confirmed by echocardiography. Four of them had diffuse cutaneous involvement and advanced pulmonary fibrosis, whereas four had anti-nRNP antibodies and mild or no fibrosis. Septal hypertrophy was detected in 12 patients, 10 of whom had limited cutaneous involvement and six had anti-nRNP antibodies. Diffuse or regional LV wall hypokinesia was seen in seven patients, of whom six had anti-Scl70 antibodies and five had diffuse cutaneous involvement. Six patients with cor pulmonale, five with abnormal LV wall motion, and three with septal hypertrophy died during the follow-vp period, Autopsy revealed LV hypertrophy in three with septal hypertrophy, disarray and irregular hypertrophy of myocytes in one of them, and extensive myocardial fibrosis in one with diffuse LV wall hypokinesia. Conclusions Septal hypertrophy and cor pulmonale caused by pulmonary vasculopathy were associated with limited cutaneous involvement or anti-nRNP antibodies, whereas abnormal LV wall motion and cor pulmonale caused by advanced pulmonary fibrosis were mostly seen in patients with diffuse cutaneous involvement or anti-Scl70 antibodies.