PMC5339528_01
(a) A mask-like face and (b) digital pitting scarring on the right index finger.
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On examination, although her vital signs were normal, a mask-like face (Figure 1(a)) and moderate thickening and induration of skin on the fingers, hands, forearms, feet and lower legs were observed, and her modified Rodnan skin score was 23. Digital pitting scarring was observed on the right index finger (Figure 1(b)).
Needle electromyography of the biceps brachii muscle. Polyphasic, short duration, and low-amplitude patterns were observed during voluntary contraction.
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Magnetic resonance imaging (MRI) of the lower legs did not show any significant skeletal muscle inflammation; however, needle electromyography revealed myopathic changes (Figure 2).
Hematoxylin-Eosin (H&E) staining of the biceps brachii muscle. Muscle fiber necrosis and regeneration and mild inflammatory cell infiltration in the perivascular region of perimysium can be observed.
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Further, muscle biopsy of the triceps brachii revealed mild perivascular inflammatory cells infiltration, muscle fiber necrosis, and regeneration (Figure 3).
Nine-week time course after the initiation of treatment. Therapeutic agents, symptoms, creatine kinase (CK, shown as open black circles in graph (a)), N-terminal pro b-type natriuretic peptide (NT-proBNP, shown as open gray circles in graph (a)), left ventricular posterior wall end diastole, percent of normal (LVPWD% N, shown as closed black triangles in graph (b)) and left ventricular diastolic diameter (LVDd, shown as closed gray triangles in graph (b)), and Tei index showing left ventricular diastolic and systolic function, calculated as [isovolumic contraction time + isovolumic relaxation time]/[left ventricular ejection time] (Tei index, shown as open squares in graph (c)) are shown. Cardiac dysfunction and exacerbation of myositis occurred abruptly and were resolved after methylprednisolone pulse therapy in combination with palliative therapies for heart failure.
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Initial treatment was started with oral prednisolone (PSL) 1 mg/kg/day (30 mg/day) and methotrexate (MTX) 10 mg/m2/week (Figure 4). Surprisingly, not only had her pericardial effusion disappeared, but also myocardial performance significantly recovered within a further two to three weeks (Figure 4).
Time course of 12-lead electrocardiogram tracings; (1) at the time of admission; (2) at the onset of heart failure; and (3) during the recovery period (4 weeks after the onset of heart failure). A right axis shift, widespread low voltage, and left ventricular hypertrophy were observed at the onset of heart failure and subsequently resolved.
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Her electrocardiogram (ECG) showed a right axis shift and widespread low voltage (Figure 5).
(a, b) Parasternal echocardiography at the onset of heart failure. (a) Short axis view showing pericardiac effusion (red arrow) and all-round ventricular hypertrophy. (b) Motion (M) mode through the left ventricle. The LVDd was 35 mm, left ventricular posterior wall systole (LVDs) 27.6 mm, LVPWD 12.6 mm (196% of normal), and interventricular septal end diastole (IVSd) 13.3 mm (211% of normal). Also, the calculated LVEF was 55%. These findings suggest that diastolic dysfunction due to biventricular hypertrophy resulted in heart failure. (c, d) Parasternal echocardiography 7 days after the initiation of steroid pulse therapy. The LVDd was 40 mm, left ventricular posterior wall systole (LVDs) 23 mm, LVPWD 8.7 mm (133% of normal), and interventricular septal end diastole (IVSd) 10 mm (158% of normal). Also, the calculated LVEF was 80%. Biventricular hypertrophy was improving although still residual, and cardiac contractility was significantly improved.
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The mitral inflow pattern showed diastolic dysfunction resulting in reduced cardiac output (Figure 6).