Pulmonary vascular disease (PVD) in patients with Sjögren syndrome - a prospective cross-sectional study

Background: Sjogren syndrome may be complicated by pulmonary hypertension (PH). However, there are no data available from prospective studies regarding its prevalence. Aims: We aimed to assess the prevalence of PVD including PH, borderline mPAP (20-24mmHg) and exercise PH (EPH) in patients with primary and secondary Sjogren syndrome. Methods: Consecutive patients with Sjogren syndrome underwent echocardiography at rest and during exercise. They were assigned to a low-, intermediate- or high-risk group according to their resting SPAP and their mPAP/CO slope during exercise. In high-risk patients (SPAP≥38mmHg, suspected PH) right heart catheterization (RHC) was suggested. Intermediate-risk patients (SPAP 30-37mmHg [suspected borderline mPAP] or exerciseSPAP≥46mmHg + TPR>3WU [suspected EPH]) RHC was advised in case of symptoms or significantly decreased peakVO2. Results: 86 patients were screened (female N=81, age 58±10yrs, primary Sjogren N=46). N=6 patients had a resting SPAP≥38mmHg, all of them meeting criteria for suspected EPH. N=9 and N=18 fulfilled criteria for suspected borderline mPAP and/or EPH, respectively. RHC was performed in 10 patients. EPH was diagnosed in N=8, borderline mPAP in N=1. No patient had PH. Patients with suspected EPH were older (64±10 vs 55±10, p Conclusions: In this first prospective study evaluating the prevalence of PVD in Sjogren syndrome, 0/86 patients had manifest PH, N=8 had EPH and N=1 had mild PAP elevation. These findings were associated with older age and diastolic LV-dysfunction.