PULMONARY ARTERIAL HYPERTENSION IN THE SETTING OF SJOGREN'S SYNDROME

Abstract

Purpose: Determine the hemodynamic and echocardiographic profile of patients with primary or secondary Sjogren's Syndrome (SS) referred for right heart catheterization for suspected pulmonary hypertension (PH). Methods: Single center cohort study of all patients with SS and suspected PH who underwent right heart catheterization between 1/1/1995 - 1/31/2014. Pulmonary arterial hypertension (PAH) was defined as a mean pulmonary artery pressure (mPAP) >= 25 mmHg, pulmonary artery wedge pressure (PAWP) < 15 mmHg, and pulmonary vascular resistance (PVR) > 3 Wood Units. Data reported as mean +/- standard deviation. Results: Of 5420 patients with Sjogren's Syndrome evaluated between 1995 and 2014, 100 had evidence of right ventricular systolic pressure (RVSP) > 40 mmHg on transthoracic echocardiography. Of those 100 patients, 40 underwent right heart catheterization (RHC); and 36 (90%) had mPAP >= 25 mmHg. Of those 36 all-female patients, the average age was 65 +/- 9 years. 20 (56%) had primary SS, and 16 (44%) had secondary SS. An underlying lung disorder was present in 13 (36%), with five carrying a diagnosis of interstitial lung disease. A left ventricular ejection fraction (EF) < 45% was present in 14 (39%). Transthoracic echocardiography for the 36 patients revealed a right atrial pressure (RAP) of 13 +/- 4 mmHg and a mean RVSP of 72 +/- 21 mmHg. Moderate-severe right atrial enlargement was present in 21 (58%) with moderate-severe right ventricular enlargement present in 21 (58%) also. 19 (53%) had