Haemosiderin-laden sputum macrophages for diagnosis in pulmonary veno-occlusive disease

PLoS One. 2014 Dec 12;9(12):e115219. doi: 10.1371/journal.pone.0115219. eCollection 2014.


Aims: 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.

Methods and 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.

Conclusion: 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Case-Control Studies
  • Female
  • Hemosiderin / metabolism*
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / metabolism
  • Macrophages / metabolism*
  • Male
  • Middle Aged
  • Pulmonary Veno-Occlusive Disease / diagnosis*
  • Pulmonary Veno-Occlusive Disease / metabolism
  • Sputum / cytology
  • Sputum / metabolism*


  • Hemosiderin

Grant support

Dr. Breitenstein received educational funding from Actelion Switzerland. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.