title: Clinical case: Combined pulmonary fibrosis and emphysema with pulmonary hypertension--clinical management.
abstract: BACKGROUND
  Combined idiopathic pulmonary fibrosis (IPF) with pulmonary emphysema (CPFE) is a syndrome with a characteristic presentation of upper lobe emphysema and lower lobe fibrosis. While CPFE is a strong determinant of secondary precapillary pulmonary hypertension (PH), there is limited evidence regarding the management of patients with CPFE and PH.
  
  
  METHODS
  A 63 year-old male presented in 2006 with dyspnoea on exertion having quit smoking in 2003. Clinical examination, together with high resolution computed tomography, bronchoalveolar lavage, and echocardiographic assessments, suggested a diagnosis of CPFE without PH. In 2007, the patient received intravenous cyclophosphamide, N-acetylcysteine, and short-term anticoagulation treatment. Due to remission of acute exacerbations, the patient received triple combination therapy (prednisone, N-acetylcysteine and azathioprine). Upon progressive clinical worsening, long-term supplemental oxygen therapy was initiated in 2009. Repeated right heart catheterisation in 2011 confirmed PH and worsening pulmonary haemodynamics, and off-label ambrisentan therapy was initiated. Dyspnoea remained at follow-up, although significant haemodynamic improvement was observed.
  
  
  CONCLUSIONS
  CFPE is a distinct but under-recognized and common syndrome with a characteristic presentation. Further studies are needed to ascertain the etiology, morbidity, and mortality of CPEF with or without PH, and to evaluate novel management options.
authors.affiliation: Hospices Civils de Lyon, Hôpital Louis Pradel, Service de pneumologie - Centre de référence national des maladies pulmonaires rares, Université Claude Bernard Lyon, Lyon, France. vincent.cottin@chu-lyon.fr
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
mesh_terms: D000111:Acetylcysteine / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D019468:Disease Management; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010102:Oxygen Inhalation Therapy; D010666:Phenylpropionates / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D011656:Pulmonary Emphysema / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D011658:Pulmonary Fibrosis / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D011724:Pyridazines / Q000627:therapeutic use; D011859:Radiography; D012907:Smoking
chemical_list: D010666:Phenylpropionates; D011724:Pyridazines; D011239:Prednisolone; C467894:ambrisentan; D001379:Azathioprine; D000111:Acetylcysteine