In September 2015, a 60 year-old woman with known history of rheumatoid arthritis (rheumatoid factor >20, cyclic citrullinated peptide antibodies >300, elevated c-reactive protein and erythrocyte sedimentation rate, synovitis in 8 small joints) and co-existing pulmonary fibrosis with chronic type 1 respiratory failure was urgently transferred to the Respiratory Intensive Care Unit (RICU) due to life-threatening hypoxia. Assessment of vital signs revealed a respiratory rate of 35 breaths/min, a heart rate of 110 bpm and a saturation of 85%, receiving maximal flow of oxygen through a non-rebreather mask. Blood gas analysis showed pH of 7.47, pO2 of 50 mm Hg, and PCO2 of 36 mm Hg. Physical examination revealed crackles on both lungs, a prominent pulmonic compound of the second heart sound, and leg edema. Electrocardiogram showed right axis deviation, pulmonary P waves, and rSR' in V1. Hematological and biochemical tests showed: leukocytosis (16.02 k/muL -80% neutro), Hct at 49.9%, Hb of 16.6 g/dL, normal procalcitonin but elevated C-reactive protein of 3.250 mg/dl, normal troponin, and elevated b-type natriuretic peptide levels. Heart echocardiography revealed right heart enlargement, tricuspid valve regurgitation, and an estimated right ventricular systolic pressure (RVSP) of 68 mm Hg. The left heart maintained normal dimensions and systolic function, but the intraventricular septum was flattened forming a D-shaped left ventricle. The patient underwent pulmonary function tests indicating moderate restrictive lung disease with a FVC of 63% pred, TLC of 50.6% pred and a diffusing capacity for carbon monoxide (DLCO) of 19% pred. Thoracic high resolution CT revealed modest honeycombing, ground-glass opacities with fibrotic compound, traction bronchiectasis, and thickening of interlobular septa (illustrated in Image 1). A ventilation perfusion scan was urgently performed and showed normal perfusion. A right heart catheterization confirmed precapillary pulmonary hypertension [PAP (s/d/m):74/35/51 mm Hg, PAWP: 12 mm Hg, Right Atrial Pressure: 11 mm Hg, Cardiac Index: 1.6 L/min/m2, SvO2: 64%, PVR: 13 Wood units, SaO2: 89%]. Early upfront combination therapy was planned for the patient and she was immediately treated with inhaled iloprost six times a day and tb sildenafil 20 mg tqd combined with her previous treatment of iv furosemide and per os methylprednisolone. Her oxygenation status gradually improved. By Day 10, she was able to maintain a pO2 of 58 mm Hg with 5 lt/min through nasal cannula. She was discharged on Day 15 with further improvement of oxygenation. At the one-year follow up she was stable under oxygen therapy in functional class NYHA III [New York Heart Association class III].