Analogously to the effort made in the field of interstitial lung disease with the recent introduction of the concept of interstitial pneumonia with autoimmune features[32] we propose that a population of PAH patients show autoimmune features which may rely undetected at a first examination and which require a multidisciplinary expertise and the mandatory performance of ANA testing and capillaroscopy. Here, BTG3 is linked to pulmonary arterial hypertension.