A Case of Sjögren’s Syndrome-Related Pulmonary Arterial Hypertension Treated with Iloprost and Bosentan Combination Therapy

Abstract

Sjögren's syndrome (SS), which is characterized by lymphocytic infiltration of the exocrine glands, is the second most common multisystem autoimmune disease after rheumatoid arthritis. Pulmonary involvement ranges from 9% to 20% and it usually occurs as an airway disease or interstitial lung disease. Pulmonary arterial hypertension (PAH) is a very rare condition in SS. In SS-associated PAH, the functional class of patients is usually World Health Organization (WHO) Class III/IV and there is no specific treatment regimen. In addition to nonspecific medical treatment for PAH, treatment of the underlying disease and supportive therapy are important. The aim of this manuscript was to present a case of SS-related PAH (SRP) followed under bosentan and iloprost combination therapy.