TY - JOUR T1 - Pulmonary arterial hypertension preceding idiopathic pulmonary fibrosis in a <em>BMPR2</em> mutation positive patient JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 147 LP - 149 DO - 10.1183/09059180.00005513 VL - 23 IS - 131 AU - Aniek J. Raamsteeboers AU - Harm Jan Bogaard AU - Anton Vonk Noordegraaf Y1 - 2014/03/01 UR - http://err.ersjournals.com/content/23/131/147.abstract N2 - To the Editor:When pulmonary hypertension (PH) develops in a patient with idiopathic pulmonary fibrosis (IPF) it is usually categorised in World Health Organization (WHO) group III: PH associated with chronic lung disease and/or hypoxia [1]. Given the fact that pulmonary arterial hypertension (PAH) (WHO group I) and IPF are both rare and fatal diseases, their manifestation in a single patient is unlikely to occur by chance. Here, we describe for the first time the concurrent development of IPF and PH in a patient with a bone morphogenetic protein receptor type 2 (BMPR2) mutation.An 80-year-old female presented with fever, chills, shortness of breath, chest pain and a decline in exercise tolerance. She had been diagnosed 3 years before with hereditary PAH (HPAH) associated with a BMPR2 mutation (c.637C&gt;T; p.Arg213X (reference sequence NM_001204)), for which she received ambrisentan, sildenafil and diuretic treatment. Right heart catheterisation at the time of HPAH diagnosis had shown a mean pulmonary artery pressure of 42 mmHg and a pulmonary arterial wedge pressure of 2 mmHg. Pulmonary function tests had shown a vital capacity of 2.7 L (139%) and a forced expiratory volume in 1 s of 2.3 L (144%). She was a former smoker and denied the … ER -