Pulmonary Arterial Hypertension Associated With Primary Sjögren’s Syndrome: An Unusual Association

Pulmonary arterial hypertension (PAH) is an increasingly recognised clinical entity that is associated with connective tissue disease (CTD) in up to one quarter of all diagnoses. Sjögren’s syndrome (SS) is a chronic autoimmune disease characterised by ocular and oral dryness resulting from lacrimal and salivary gland dysfunction. Additionally, SS may involve virtually any organ system and, as a result, the disease is characterised by pleomorphic clinical manifestations. However, SS-PAH reports are scarce, and the area remains insufficiently studied. We present a case of a 75-year-old female with a new diagnosis of PAH and SS. LEARNING POINTS: 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.