Pulmonary Fibrosis

Pulmonary arterial hypertension (PAH) is defined by a pathological increased pressure in the pulmonary vasculature and is associated with a very poor prognosis with 5-year survival rates of about 30 % in untreated disease. Up to now, no (medicinal) cure for PAH is available and only lung transplantation remains as an ultima ratio.

Histomorphological hallmarks of advanced PAH are so called plexiform and concentric lesions (PL, CL) and consist of complex vascular rearrangements. It is under debate, whether these are a mere consequence of increased intravascular pressure or actively participate in disease progression. It is generally accepted that PL are misguided neo-vascularisations, resulting from a dysbalance between endothelial apoptosis/necrosis, followed by circumscribed endothelial proliferation.

Studies on human PAH are challenging due to limited availability of fresh lung tissue. Therefore an appropriate (animal) model, which closely mimics the pathophysiologic changes seen in human disease, is required. To this end, we collaborate with specialized research centers around the globe.

We are interested to elucidate the mechanisms controlling the progredient vascular remodelling in human PAH. Together with our collaboration partners we aim to identify optimal animal models of PH which closely mimic the pathophysiologic changes seen in human disease and use these to “reverse engineer” test findings from human patients in functional studies.

Copyright: Prof. D. Jonigk, Pathologie, MHH