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

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

Heidi Lederer  1 , Bettina Muggli  2 , Rudolf Speich  1 , Ula Treder  1 , Hans Stricker  3 , Jeroen Goede  4 , Silvia Ulrich  1 , Simon F Stämpfli  2 , Alexander Breitenstein  5
Affiliations

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

Heidi Lederer et al. PLoS One. .

Abstract

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.

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Conflict of interest statement

Competing Interests: Dr. Breitenstein received educational funding from Actelion Switzerland. This does not alter the authors' adherence to PLOS policies on data sharing and materials.

Figures

Figure 1
Figure 1. Description of the Golde score calculation in sputum macrophages.
Figure 2
Figure 2. Haemosiderin-laden macrophages are significantly (P<0.05) more present in sputum from PVOD patients as compared to the others (IPAH, CTEPH and SAPH, respectively).
Figure 3
Figure 3. Histologic specimen from a patient suffering from PVOD who underwent lung transplantation.
Intimal thickening and fibrous obstruction of septal veins and preseptal venules (asterixis) as well as features of pulmonary capillary hemangiomatosis with proliferation of dilated and congested capillaries as well as dilated lymphatic (left upper and right lower part of the figure; arrowhead). Alveoli with haemosiderin-laden macrophages of Golde score 3–4 (arrow) are clearly visible. Haemosiderin-negativ macrophages are difficult to be identified. Golde score from the tissue was 304, as compared to 252 in the sputum.
See this image and copyright information in PMC

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