Pulmonary Arterial Hypertension Associated With Primary Sj ö gren's Syndrome: An Unusual Association.

SS is a chronic autoimmune disease that may involve virtually any organ system, representing a rare cause of PAH.The exclusion of SS as a possible diagnosis is needed before a diagnosis of idiopathic PAH can be made.Routine screening of PAH is recommended in SS patients and future studies should clarify the optimal management of these patients, including immunosuppressive therapy.