Endothelial to mesenchymal transition (EndoMT) in the pathogenesis of Systemic Sclerosis-associated pulmonary fibrosis and pulmonary arterial hypertension. Myth or reality?

Systemic Sclerosis (SSc) is a systemic autoimmune disease characterized by progressive fibrosis of skin and multiple internal organs and severe functional and structural microvascular alterations. SSc is considered to be the prototypic systemic fibrotic disorder. Despite currently available therapeutic approaches SSc has a high mortality rate owing to the development of SSc-associated interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH), complications that have emerged as the most frequent causes of disability and mortality in SSc. The pathogenesis of the fibrotic process in SSc is complex and despite extensive investigation the exact mechanisms have remained elusive. Myofibroblasts are the cells ultimately responsible for tissue fibrosis and fibroproliferative vasculopathy in SSc. Tissue myofibroblasts in SSc originate from several sources including expansion of quiescent tissue fibroblasts and tissue accumulation of CD34+ fibrocytes. Besides these sources, myofibroblasts in SSc may result from the phenotypic conversion of endothelial cells into activated myofibroblasts, a process known as endothelial to mesenchymal transition (EndoMT). Recently, it has been postulated that EndoMT may play a role in the development of SSc-associated ILD and PAH. However, although several studies have described the occurrence of EndoMT in experimentally induced cardiac, renal, and pulmonary fibrosis and in several human disorders, the contribution of EndoMT to SSc-associated ILD and PAH has not been generally accepted. Here, the experimental evidence supporting the concept that EndoMT plays a role in the pathogenesis of SSc-associated ILD and PAH will be reviewed

A precessing jet model for the PN K 3-35: simulated radio-continuum emission

The bipolar morphology of the planetary nebula (PN) K 3-35 observed in radio-continuum images was modelled with 3D hydrodynamic simulations with the adaptive grid code yguazu-a. We find that the observed morphology of this PN can be reproduced considering a precessing jet evolving in a dense AGB circumstellar medium, given by a mass loss rate \dot{M}_{csm}=5x10^{-5}M_{\odot}/yr and a terminal velocity v_{w}=10 km/s. Synthetic thermal radio-continuum maps were generated from numerical results for several frequencies. Comparing the maps and the total fluxes obtained from the simulations with the observational results, we find that a model of precessing dense jets, where each jet injects material into the surrounding CSM at a rate \dot{M}_j=2.8x10^{-4} {M_{\odot}/yr (equivalent to a density of 8x10^{4} {cm}^{-3}, a velocity of 1500 km/s, a precession period of 100 yr, and a semi-aperture precession angle of 20 degrees agrees well with the observations.Comment: 6 pages, 4 figures, accepted to MNRA