title: Haemosiderin-laden sputum macrophages for diagnosis in pulmonary veno-occlusive disease.
abstract: OBJECTIVE
  Pulmonary veno-occlusive disease (PVOD) is a rare condition of pulmonary arterial hypertension (PAH), in which post-capillary veins are affected. Since the therapeutic approach in PVOD differs from other forms of PAH, it is crucial to establish the diagnosis. Due to the fact that affected patients are often hemodynamically unstable, minimal invasive procedures are necessary for the diagnostic work-up. Chronic alveolar haemorrhage has been observed during bronchoalveolar lavage in PVOD cases. This study therefore investigates whether signs of alveolar haemorrhage can also be found in the sputum of these patients.
  
  
  RESULTS
  Six patients suffering from PVOD were included in this analysis. As controls, patients with idiopathic PAH (n = 11), chronic thromboembolic PH (n = 9) and with sclerodermia-associated PH (n = 10) were assessed. Sputum from every patient was obtained by a non-invasive manner. The amount of haemosiderin-laden macrophages was determined using the Golde score. There were statistically significant more haemosiderin-laden macrophages in the sputum of patients suffering from PVOD as compared to the other groups (P<0.05). Assuming a cut-off of 200 on the Golde score, all of the 6 PVOD patients surpassed this value compared with only 1 out of the 30 cases with precapillary PH. Thus, sensitivity and specificity with respect to the diagnosis of PVOD was 100% and 97%, respectively.
  
  
  CONCLUSIONS
  The content of haemosiderin-laden macrophages in the sputum of patients suffering from PVOD is significantly higher as compared to other forms of PH and may be useful in the non-invasive diagnostic work-up of these patients.
authors.affiliation: Zurich Pulmonary Hypertension Program, Clinic for Internal Medicine, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Heidi
authors.initials: H
authors.lastname: Lederer
authors.affiliation: Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Bettina
authors.initials: B
authors.lastname: Muggli
authors.affiliation: Zurich Pulmonary Hypertension Program, Clinic for Internal Medicine, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Rudolf
authors.initials: R
authors.lastname: Speich
authors.affiliation: Zurich Pulmonary Hypertension Program, Clinic for Internal Medicine, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Ula
authors.initials: U
authors.lastname: Treder
authors.affiliation: Ospedale La Carità, Via all' Ospedale, Locarno, Switzerland.
authors.forename: Hans
authors.initials: H
authors.lastname: Stricker
authors.affiliation: Department of Hematolgy, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Jeroen
authors.initials: J
authors.lastname: Goede
authors.affiliation: Zurich Pulmonary Hypertension Program, Clinic for Internal Medicine, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Silvia
authors.initials: S
authors.lastname: Ulrich
authors.affiliation: Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Simon F
authors.initials: SF
authors.lastname: Stämpfli
authors.affiliation: Zurich Pulmonary Hypertension Program, Clinic for Internal Medicine, University Hospital Zurich, Zurich, Switzerland; Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland; Department of Cardiology, St Bartholomew's Hospital, Barts Health NHS Trust, London, United Kingdom.
authors.forename: Alexander
authors.initials: A
authors.lastname: Breitenstein
mesh_terms: D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D005260:Female; D006485:Hemosiderin / Q000378:metabolism*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000378:metabolism; D008264:Macrophages / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis* / Q000378:metabolism; D013183:Sputum / Q000166:cytology / Q000378:metabolism*
chemical_list: D006485:Hemosiderin