A 45-year old female originally presented in January 2001 with a 25 pack-year history of tobacco use, a 2-year history of dyspnoea on exertion, fatigue and polycythaemia. Right heart catheterisation showed a pulmonary artery pressure of 54/26 mmHg (mean 37) with a cardiac index of 2.3 L·min-1·m-2. An open lung biopsy was performed and the samples from histopathological examination are shown in figure 1.
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