Clinical features and endothelin receptor antagonist in primary Sjögren's syndrome with pulmonary arterial hypertension.

The article discusses the clinical features and endothelin receptor antagonist in Sjögren's syndrome with pulmonary arterial hypertension (PAH). PAH is an unusual complication of primary Sjögren's syndrome which is caused by an immune-mediated mechanism. Some of the effective regimen to treat PAH are Bosentan and aggressive immunosuppressive drug therapy.