Systemic sclerosis (SSc) is a rare chronic connective tissue disease of unknown etiology, characterized by microvasculopathy, immunological abnormalities, and fibrosis of the skin and visceral organs. 1,2 SSc is more common in women than men (ratio between 4:1 and 14:1), usually affecting middle-aged adults. 3,4 It has a higher mortality rate than any other rheumatic disease. 5,6 Epstein-Barr virus (EBV), a lymphotropic virus that chronically affects 90% of the human population, has been proposed as a possible environmental trigger of SSc. [7][8][9][10][11][12] Topoisomerase autoantibodies, a specific marker for SSc, are shown to be produced by EBV-transformed B cells from healthy