Adult-onset Still's disease and pulmonary arterial hypertension

Summary Pulmonary arterial hypertension (PAH) occurs with several rheumatologic diseases, however, it is rarely reported with Adult-onset Still's disease (AOSD). Pulmonary arterial hypertension is a major cause of morbidity and mortality. We describe a patient with AOSD who developed the characteristic clinical and hemodynamic findings of idiopathic PAH with persistent hypoxemia. A remission was obtained with immunosuppressive treatment without addition of prostaglandins, endothelin antagonists or phosphodiesterase 5 inhibitors.