We wish to share our experience in an unusual case presentation of pulmonary hypertension. A 48-year-old Caucasian female with a history of hypothyroidism and smoking presented with progressive dyspnoea on exertion for 3 years, markedly worse during the past 6 months. She also had arthralgias, Raynaud’s phenomena, night sweats and a 25-pound weight loss over 6 months. Chest computed tomography scan showed diffuse mediastinal lymphadenopathy. Lymph node biopsy showed non-necrotising epithelioid granulomas compatible with sarcoidosis. Symptoms worsened despite treatment with prednisone.

Pulmonary function tests showed mild restrictive disease, reduced diffusion capacity and desaturation during a 6-min walk. Echocardiogram showed a severely dilated and hypokinetic right ventricle. Right heart catheterisation showed pulmonary artery pressure 79/38 mm Hg (mean 46 mm Hg), cardiac output 6.7 …