%0 Journal Article
%T Pulmonary Arterial Hypertension in Patients with Primary Sj£¿gren¡¯s Syndrome
%A Senol Kobak
%A Sezai Kalkan
%A Bahad£¿r Kirilmaz
%A Mehmet Orman
%A Ertu£¿urul Ercan
%J Autoimmune Diseases
%D 2014
%I Hindawi Publishing Corporation
%R 10.1155/2014/710401
%X Introduction. Primary Sj£¿gren¡¯s syndrome (pSS) is an autoimmune epithelitis. Pulmonary arterial hypertension (PAH) is an important and severe complication, which is encountered in many collagen tissue disorders. Early diagnostic strategies are required to define it at the asymptomatic stage. Doppler echocardiography is an important, noninvasive screening test for PAH diagnosis. Objective. The aim of this present study is to define the frequency of PAH in patients with pSS and to reveal correlations with laboratory and clinical findings. Material and Methods. A total of 47 patients, who were diagnosed with pSS according to American-European Study Group criteria were enrolled in the study. After all patients were evaluated clinically and by laboratory tests, Doppler echocardiography was performed in the cardiology outpatient clinic. Systolic pulmonary artery pressure (SPAP) >30£¿mm£¿Hg values, which were measured at the resting state, were accepted as significant for PAH. Results. Forty-seven patients with pSS were included in the study. The mean age of patients was 48 years and the mean disease duration was 5.3 years. PAH was defined in 11 of the 47 patients (23.4%). The SPAP value was over 35£¿mm£¿Hg in 5 out of 11 patients, whereas six patients had SPAP measuring 30¨C35£¿mm£¿Hg. While pulmonary hypertension was related with earlier age and shorter duration of disease ( ), there was no statistically significant correlation between SPAP increase and clinical findings ( ). Conclusion. We have defined high PAH frequency in patients with pSS. Since there are different data in the literature, it is obvious that large scale, multicentre studies are required. 1. Introduction Pulmonary arterial hypertension (PAH), which may progress with a fatal outcome, is characterized by increased right ventricular pressure and pulmonary vascular resistance. It may result in cardiac failure, pulmonary edema, arrhythmia, and/or sudden death [1]. It may develop secondary to an underlying disease (cardiac and lung diseases, connective tissue diseases, etc.) along with primary PAH with an unknown etiology [2]. PAH is a serious and sometimes fatal complication in various connective tissue diseases (like scleroderma, mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), etc.) [3¨C5]. It may be encountered with an underlying interstitial fibrosis or direct proliferative vascular involvement without any parenchymal diseases [6]. Different pathogenetic mechanisms have been discussed in PAH, which is observed among connective tissue diseases.
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