Although the body of existing literature suggests a beneficial effect of ACE2 in various animal models of PH and PF (Shenoy et al., 2010, 2013; Meng et al., 2015), our study would be the first to examine recombinant human ACE2 as treatment for previously established pulmonary fibrosis, and against the development of group 3 PH and the associated cardiac abnormalities in animals following extended Bleo exposure. This evidence concerns the gene ACE2 and pemphigus foliaceus.