Systemic Sclerosis (SSc) is a rare autoimmune disease that is characterized by a progressive skin fibrosis, an obliteration of the microvasculature and an exaggerated extracellular matrix deposition, which lead to a multisystemic dysfunction. Various pathogenetic mechanisms were described. The lack of a successful therapy make SSc a disease with a poor prognosis. The intravenous immunoglobulin (IVIG) has been used for a long time in different autoimmune diseases, and firstly used in SSc patients in 2000. IVIG has multiple non-specific mechanisms of action and, beyond an impressive improvement in muscle symptoms, a French nationwide cohort demonstrated that IVIG ameliorates the skin disease and systemic inflammation, and helps the daily dose corticosteroid's tapering at the end of the treatment. The benefits on gastrointestinal symptoms of IVIG was reported by a recent English article, in which the patients consistently reported a decrease in the gastro-esophageal reflux disease symptoms and their frequencies. The impact on the lung involvement still remains unclear. One of the advantages of IVIG is its safe profile. Few adverse effects were reported and most of them are mild, and can be managed and usually they do not relapse. Harmful effects were described, but they can be avoid with cautious and judicious use of this therapy.

系统性硬化症（SSc）是一种罕见的自身免疫性疾病，其特征是进行性皮肤纤维化，微脉管系统的闭塞和细胞外基质沉积的增加，从而导致多系统功能障碍。描述了各种致病机制。缺乏成功的治疗方法使SSc成为预后不良的疾病。静脉免疫球蛋白（IVIG）已在多种自身免疫疾病中使用了很长时间，并于2000年首次在SSc患者中使用。IVIG具有多种非特异性作用机制，并且在法国全国范围内，除肌肉症状显着改善外，证明IVIG可改善皮肤疾病和全身性炎症，并在治疗结束时帮助每日剂量的皮质类固醇逐渐减少。最近的一篇英语文章报道了IVIG对胃肠道症状的益处，其中患者一贯报道胃食管反流疾病症状及其频率减少。对肺部受累的影响仍不清楚。IVIG的优点之一是它的安全性。几乎没有不良反应的报道，而且大多数是轻度的，可以控制并且通常不会复发。描述了有害作用，但谨慎和明智地使用此疗法可避免有害作用。几乎没有不良反应的报道，而且大多数是轻度的，可以控制并且通常不会复发。描述了有害作用，但谨慎和明智地使用此疗法可以避免有害作用。几乎没有不良反应的报道，而且大多数是轻度的，可以控制并且通常不会复发。描述了有害作用，但谨慎和明智地使用此疗法可以避免有害作用。