Is the heart affected in primary Sjögren's syndrome? An echocardiographic study

To evaluate whether patients with primary Sjögren's syndrome without overt cardiac disease have echocardiographic abnormalities and their relation with clinical and laboratory data.

One hundred and seven consecutive patients with primary Sjögren's syndrome and 112 healthy controls, matched for age and gender, underwent complete echocardiographic study.

Thirty-two patients had mitral valve regurgitation (p<0.001) whereas tricuspid and aortic valve regurgitation were, also, more frequent in the patient group (p=0.022 and p=0.007 respectively). In multivariate analyses, low C4 levels of complement and age were strong predictors of mitral valve regurgitation whereas age was predictor of aortic valve regurgitation. Tricuspid valve regurgitation was associated with pulmonary hypertension. Clinically silent pericardial effusion, found in 9 patients (p=0.008), was associated with cryoglobulinemia and primary biliary cirrhosis. Twenty-four patients had pulmonary hypertension (p<0.001) whereas hypocomplementemia and cryoglobulinemia were strong predictors of pulmonary artery systolic pressure. The analyses reveal that easy fatigability was associated with pulmonary hypertension and low C4 levels. The patients' left ventricular mass index differed significantly from the controls (108.9+/-17.21 gm(-2) vs. 85.8+/-6.73 gm(-2), p<0.001) and was associated with palpaple purpura and anti-Ro/SSA. From the diastolic function indices only the left ventricular isovolumic relaxation time differed significantly among patients and controls.

Valvular regurgitation, pericardial effusion, pulmonary hypertension and increased left ventricular mass index occur with disproportionately high frequency in patients with primary Sjögren's syndrome and no clinically apparent heart disease. Thus echocardiographic studies may need to be performed in these patients especially when palpable purpura, antibody reactivity and low C4 levels are present.