What is it about?
By means of FACS analysis from circulating blood, PAH patients were compared to matched controls. There were singificant differences concerning the number of lymphocytes and the number of CD133 positive progenitor cells. However, within the CD133 positive cells, some were subgroups were significantly increased and others were significantly decreased. In the tissue of explanted end-stage PAH lungs, CD133 positive cells were not associated to the vascular structures but rather to epithelial cells that might contribute to the regenerative pool in the lung.
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Why is it important?
PAH is a disease of unknown origin. Our data suggest that circulating cells contribute to the pathology of pulmonary vascular remodeling but that the interactions between the circulating cells and the resident cells are not well understood.
Perspectives
This work may open new pathways of understanding pulmonary vascular remodeling and identifying new therapeutic targets for treatment of PAH.
Horst Olschewski
Medical University of Graz
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This page is a summary of: CD133+cells in pulmonary arterial hypertension, European Respiratory Journal, April 2016, European Respiratory Society (ERS),
DOI: 10.1183/13993003.01523-2015.
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