Although anti-CXCR3 and/or anti-CXCR4 antibodies have been found to be increased in SSc, with anti-CXCR3 autoantibodies from SSc patients preferentially binding to intracellular epitopes instead of extracellular epitopes as in healthy individuals, their pathological functional activity remains to be elucidated [4,76,77,96]. Here, CXCR4 is linked to systemic sclerosis.