The aim of the study was to determine for the first time the response of systemic sclerosis (SSc) patients to treatment with intravenous immunoglobulin (IVIg). Three patients with progressive and rapidly deteriorating disease (mainly affecting the skin) were planned to receive six monthly courses of high-dose IVIg (2 g/kg). All had a thorough physical examination, clinical evaluation by the modified Rodnan total skin thickness score, and measurement of the titres of PM-Scl antibodies before and after the treatment, and before and after each treatment course. Two of the three patients received six IVIg courses as planned and no adverse effects or disease progression occurred during the therapy. The third patient received three courses, after which he developed renal failure and later died of sepsis. All three patients had a large decrease in their skin score after the treatment compared to that before the treatment. No modification of PM-Scl antibody titres was noted in any patient. Intravenous immunoglobulin (IVIg) may have a role in the treatment of SSc patients with rapidly deteriorating skin disease. The specific indications, as well as the safety of this treatment, should be further researched.