Objective Myocardial inflammation and fibrosis are common autopsy findings
in systemic sclerosis (SSc) and, although symptomatic cardiac involvement o
ccurs less often, current therapies remain empiric and do not prevent or mo
dify, its course. In this open, uncontrolled study we assessed the short-te
rm effects of glucocorticoid administration on myocardial performance in pa
tients with SSc in the absence of clinically overt cardiac disease.
Methods Resting radionuclide ventriculography with Tc-99m was performed bef
ore and 20 days after the administration of prednisolone, 20 mg daily, in 3
2 patients with SSc without clinically evident myocardial dysfunction at re
st; 13 and 19 patients with systemic lupus erythematosus (SLE) and rheumato
id arthritis (RA), respectively, were studied in parallel as controls.
Results The mean left ventricular ejection fraction (LVEF) value at baselin
e was 59% in the SSc group; similar values were found for the SLE (61%) and
RA (59%) groups. An impaired LVEF (i.e., < 50%) was found in 6 patients wi
th SSc and in 1 patient with SLE. Prednisolone administration resulted in a
significant percent improvement in the baseline LVEF (mean 18%, p = 0.0001
) in the SSc group; this improvement was greater in the patients with diffu
se SSc than in those with limited skin disease (27% vs 10%, p = 0.02). The
improvement was most prominent in the 6 patients with an initial impaired L
VEF: No significant improvement was observed in the SLE or RA control group
s. The linear trend between the individual baseline LVEF values in patients
with SSc and their percent changes after treatment (r(2) = 0.55 p: 0.00001
) showed that the lower the initial LVEF the greater the improvement caused
by prednisolone. The degree of LVEF improvement was also associated with t
he individual erythrocyte sedimentation rate values and serum IgG concentra
tions at baseline. Prednisolone-induced changes in LVEF were not associated
with any changes in blood pressure, heart rate, blood, plasma, or red cell
volumes.
Conclusion Glucocorticoid administration may improve myocardial performance
in some patients with SSc. Although further double-blind controlled studie
s of the long-term effects are warranted, such treatment may be useful in t
hose patients with SSc and documented low LVEF: if they are kept under care
ful observation for objective improvement.