Systemic sclerosis is a connective tissue disease with chronic vascular, autoimmune and tissue stromal progressive alterations. It is an orphan disease that is clinically characterized by two hallmarks: Raynaud’s phenomenon and skin involvement followed by a varying prevalence of internal organ involvement. There is an annual disease incidence of 19 per million and prevalence of 19–75 per 100,000. In this book of eighteen chapters, the world’s top experts provide an update on the most recent advances on pathophysiology/etiology, clinical issues, advanced diagnosis and therapy in systemic sclerosis.
