Prevalence of primary Sjogren's syndrome in patients undergoing evaluation for pulmonary arterial hypertension.

ABSTRACT:

Background

The prevalence of pulmonary arterial hypertension (PAH) in primary Sjögren's syndrome (SS) had been reported to be rare. However, recent studies using echocardiography as a screening method showed conflicting results, and the true prevalence is still unclear. Since diagnosing primary SS is difficult because of its heterogeneous nature, a number of patients with primary-SS-associated PAH may be misdiagnosed with idiopathic PAH, losing their chance to undergo immunosuppressive therapy. Therefore, we sought to elucidate the prevalence of primary SS among patients who initially present with PAH.

Methods

From our prospective institutional PAH database, 40 consecutive patients without any obvious cause of PAH at the time of PAH diagnosis were identified. We retrospectiv

SUBMITTER: Sato T 

PROVIDER: S-EPMC5953489 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature