Introduction
Systemic sclerosis (SSc)1 is a multisystem, autoimmune, connective tissue disorder that causes widespread fibrosis of the skin and internal organs. The clinical hallmarks of SSc are vascular abnormalities, excessive collagen synthesis, and high-titer autoantibody production to proteins of the nucleus and nucleolus [1-4]. Among systemic autoimmune disorders, SSc is one
of the most disabling and invalidating diseases, severely affecting the quality of life [5,6]. Recent
data have highlighted the poor efficacy of currently available drugs on skin sclerosis, interstitial lung disease, and pulmonary hypertension [7], the latter two conditions being the major causes of death in these patients. Because of the lack of effective medicines, the severity and the relatively rarity of this disease, which affects between 50 and 300 cases per million persons with an incidence ranging from 2.3 to 22.8 cases/million year [8,9], SSc is listed as an orphan by Orphanet and the European League Against Rheumatism. Very little is known about the disease’s pathogenesis, starting from early inflammatory events to fibrosis of the cutis and internal organs.