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Primary Sjögren’s syndrome (pSS) is a chronic autoimmune disease characterised by lymphocytic infiltration of the salivary and lacrimal glands.1 Similar lymphocytic infiltrates may invade visceral organs, and this results in several extraglandular manifestations.1 Among these, a clinically overt heart disease is very rare.2-4 However, recent echocardiographic studies showed that asymptomatic cardiac involvement is frequent in pSS. Thus, Rantapää-Dahlqvist and colleagues5 reported signs of present or previous pericarditis in nine of 27 (33%) pSS patients. Of the echocardiographic measurements, the right ventricular anterior wall and the left ventricular posterior wall were significantly smaller in patients with pericarditis than in those without pericardial serositis. Moreover, in the pericarditis patients, the regional fractional shortening of the left ventricle was significantly higher and the hypokinesia of the left ventricle significantly more frequent, when compared with those without pericarditis. Sclerosis of the aortic cups and a slight aortic regurgitation were seen in 11 and three patients, respectively. No patient had mitral valve prolapse or indirect signs of pulmonary hypertension. Mita et al 6 evaluated 112 patients with SS, primary in 33 and secondary in 79, by two dimensional echocardiography. They reported abnormal findings in 69 (61.6%) of the total cohort and in 55.5% of the pSS patients. In this second group, pericardial effusion was seen in 21.2%, thickening/calcification of the aortic valve in 10.3%, decrease in the diastolic descent rate of mitral valve in 6.9%, thickening/calcification of mitral valve in 3.4%, mitral regurgitation in 3.3%, and mitral prolapse in 3.2%. No pSS patient had pulmonary hypertension. Gyöngyösi et al 7 examined 64 pSS patients and showed an echogenic pericardium in 21 (33%). Pulmonary pressure was significantly higher in the patients group than in controls, probably because of interstitial lung disease. Left systolic parameters and left atrial diameter did not differ between the pSS patients and controls. On the contrary, the E:A wave ratio, the main Doppler index of left ventricular diastolic function, was abnormal in 21 of 42 (50%) patients, in 17 of whom other parameters of diastolic function were significantly changed. No correlation between the left ventricular diastolic dysfunction and presence of an echogenic pericardium was found.
We evaluated 18 female patients diagnosed as suffering from pSS according to the EEC criteria8 by M-mode and two dimensional echocardiography. The mean age (SD) was 55.3 (7.4) years (range 46–67) and the mean disease duration (SD) was 6 (4.8) years (range 6 months–20). The control group consisted of 18 age matched healthy women. No patient or control had history of cardiovascular diseases, such as arterial hypertension or ischaemic heart disease. Echocardiography was carried out with an ATL Apogee 800 instrument and normal values of the measured parameters were taken from Feigenbaum.9 Transmitral diastolic flow velocities were recorded by pulsatile Doppler method. Moreover, the left ventricular diastolic function was evaluated according to Choong.10Statistical analysis was performed using the Student’s ttest and Scheffe’s method for multiple comparison among means. The results show that only the deceleration of the E wave was significantly reduced in pSS (mean (SD)) (360 (84.02) cm/s2) compared with controls (462 (84.25) cm/s2) (p<0.0009), and remained significantly different when five subjects older than 60 years were excluded from both groups. No significant valvular disease was found in both groups. Additionally, present or previous pericarditis and pulmonary hypertension were not detected in pSS.
In conclusion, although overt heart involvement in pSS is very rare echocardiography shows an unexpectedly high frequency of cardiac manifestations, mainly pericarditis and diastolic dysfunction. These findings suggest that cardiac involvement must be included in the spectrum of extraglandular manifestations of pSS.
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