Systemic sclerosis (SSc) is an autoimmune disease which involves the skin, as well as several internal organs. Most therapies available in this disease are symptomatic. Authors present a case of diffuse SSc with progressive disease not responding to currently available treatments. Therefore a 12-month protocol of repeated plasmapheresis and high-dose intravenous immunoglobulin treatment was administered with good clinical efficacy. Apart from monitoring the clinical symptoms throughout the treatment, authors also assessed a number of humoral and cellular immunolaboratory markers in order to obtain information on the immunomodulatory effects of this combined treatment in SSc.