EDN1 and Autoimmunity: This proposition is based on the main findings of this study: (1) fibrosis in the skin and lung; (2) abnormal lung mechanics; (3) overexpression of genes which encode chains of Col I, Col III, and Col V; (4) vasculopathy with strong signals for ET-1, VEGF, and caspase-3 and (5) autoimmunity with positive ANA, in addition to anti-Col III and anti-Col IV autoantibodies.