Heart structure and function in patients with generalized autoimmune diseases: echocardiography with tissue Doppler...

Heart pathology strongly influences the course and prognosis of patients with generalized autoimmune diseases. In spite of autoimmunity being a common denominator of these diseases, systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and dermato/polymyositis (DPM) differ significantly in the pathogenesis of organ damage. The aim of the study was to compare pathologic changes in heart structure and function in these diseases by means of standard echocardiography and tissue Doppler (TDE).

Four groups were examined: 60 SSc, 60 SLE and 15 DPM patients in stable clinical conditions and 30 healthy control subjects. Echocardiography with TDE was performed with the assessment of systolic (S) and diastolic (E) velocities of mitral and tricuspid annuli.

Heart in SSc was characterized by significant diastolic left ventricular dysfunction (mitral E 8.61 +/- 2.3 cm/s vs. 12.4 +/- 3.5 cm/s in the control group; P < 0.01) with preserved systolic function (mitral S 7.85 +/- 1.5 cm/s vs. 7.95 +/- 0.9 cm/s in control group; ns). SLE and DPM resulted mainly in pathologic thickening of valvular leaflets and/or pericardium [mitral or aortic leaflets thickened in 38 (63.3%) of SLE patients, 7 (46.7%) of DPM patients; pericardium thickened in 36 (60%) of SLE patients]. Pulmonary capillary wedge pressure was elevated in SSc (13.8 +/- 3.5 mmHg) and DPM (13.2 +/- 2.5 mmHg) patients as compared to the control group (9.2 +/- 3.7 mmHg, P < 0.01). Right ventricular systolic and diastolic dysfunction was frequent irrespective of the presence or absence of pulmonary hypertension.

Echocardiography with TDE reveals characteristic pathology in different forms of generalized autoimmune diseases reflecting their different pathogenetic mechanisms. Overproduction of collagen in SSc results in diastolic left ventricular dysfunction, while generalized inflammation in SLE and DPM leads mainly to pathologic changes on valvular leaflets and/or pericardium. Interestingly, right ventricular dysfunction is common in all diseases analyzed, regardless of the presence of pulmonary hypertension. Echocardiography, preferably with TDE, could add valuable information about usually asymptomatic heart pathology in an individual patient with generalized autoimmune disease.