The etiology and pathogenesis of systemic sclerosis are still largely unknown, but a variety of humoral and cellular autoimmune phenomena have been documented. In addition, the rarity of the disease, the broad spectrum of clinical manifestations, and the relevant risk of severe complications as well as the highly variable disease course render its management a major challenge. Some immunomodulatory agents have been used, but no single agent has given a convincing proof of effectiveness, and treatment has remained largely symptomatic through recent years. Novel therapies are currently being tested and may have the potential of modifying the disease process and overall clinical outcome. Efficacy of intravenous immunoglobulins (IVIG) in different regimens (1-2 g/kg of body weight, administered over 2-5 consecutive days) has been described in a limited number of trials and small case series, showing benefits in skin, articular, and lung interstitial disease symptoms. However, studies on IVIG in systemic sclerosis still remain few, and further randomized controlled trials should be undertaken to assess their clinical effectiveness or define the optimal dosage and times of administration.