Abstract：
OBJECTIVE: Systemic sclerosis (SSc) is a highly heterogeneous disease whose treatment is based mainly on immunosuppressants, antifibrotics, and vasodilators. Intravenous immunoglobulin (IVIG) have proved effective in other autoimmune diseases. The objective of this study is to evaluate the efficacy and safety of IVIG in SSc.
METHODS: The systematic review was conducted according to the PRISMA Statement. Medline, Embase and Cochrane Library databases were searched until March 2024. We assessed the quality of included studies using the Cochrane Risk of Bias 2.0 tool (RoB 2) for randomised clinical trials and the Cochrane Risk in non-randomized studies (ROBINS-I) tool for observational studies.
RESULTS: From 1242 studies identified, 15 studies were included, of which 14 were observational studies. In total, 361 patients with SSc were included, and 295 received treatment with IVIG. Most of the studies used a dose of 2 g/kg IVIG. Ten studies, including the clinical trial, showed high risk of bias, and five had a critical risk. Skin involvement was assessed using modified Rodnan skin score, in 11 studies and the authors reported cutaneous efficacy in 9 of them. The 6 studies that assessed muscle involvement reported an improvement. Six studies reported data on gastrointestinal efficacy. Other domains such as lung and joint involvement and steroid-sparing effect were evaluated. The most frequent adverse events were mild, including headache, abdominal pain, fever, and skin rash.
CONCLUSIONS: Treatment with IVIG in SSc patients could be helpful and safe in patients with cutaneous, muscular, or digestive manifestations.
METHODS: The systematic review was conducted according to the PRISMA Statement. Medline, Embase and Cochrane Library databases were searched until March 2024. We assessed the quality of included studies using the Cochrane Risk of Bias 2.0 tool (RoB 2) for randomised clinical trials and the Cochrane Risk in non-randomized studies (ROBINS-I) tool for observational studies.
RESULTS: From 1242 studies identified, 15 studies were included, of which 14 were observational studies. In total, 361 patients with SSc were included, and 295 received treatment with IVIG. Most of the studies used a dose of 2 g/kg IVIG. Ten studies, including the clinical trial, showed high risk of bias, and five had a critical risk. Skin involvement was assessed using modified Rodnan skin score, in 11 studies and the authors reported cutaneous efficacy in 9 of them. The 6 studies that assessed muscle involvement reported an improvement. Six studies reported data on gastrointestinal efficacy. Other domains such as lung and joint involvement and steroid-sparing effect were evaluated. The most frequent adverse events were mild, including headache, abdominal pain, fever, and skin rash.
CONCLUSIONS: Treatment with IVIG in SSc patients could be helpful and safe in patients with cutaneous, muscular, or digestive manifestations.
摘要：
目的：系统性硬化症（SSc）是一种高度异质性的疾病，其治疗主要基于免疫抑制剂，抗纤维化药物,和血管扩张剂.静脉免疫球蛋白（IVIG）已被证明对其他自身免疫性疾病有效。本研究的目的是评估IVIG在SSc中的有效性和安全性。
方法：根据PRISMA声明进行系统评价。Medline,搜索Embase和Cochrane图书馆数据库，直到2024年3月。我们使用Cochrane偏差风险2.0工具（RoB2）进行随机临床试验，使用Cochrane风险非随机研究（ROBINS-I）工具进行观察性研究，评估纳入研究的质量。
结果：从确定的1242项研究中，包括15项研究，其中14项是观察性研究。总的来说,纳入了361例SSc患者，295人接受IVIG治疗。大多数研究使用2g/kgIVIG的剂量。十项研究，包括临床试验,表现出很高的偏见风险，五个有严重的风险。使用改良的Rodnan皮肤评分评估皮肤受累情况，在11项研究中，作者报告了其中9项的皮肤功效。评估肌肉受累的6项研究报告了改善。六项研究报告了胃肠道功效的数据。评估了其他领域，例如肺和关节受累以及保留类固醇的作用。最常见的不良事件是轻度的，包括头痛,腹痛,发烧,还有皮疹.
结论：在SSc患者中使用IVIG治疗可能对皮肤，肌肉，或消化表现。
方法：根据PRISMA声明进行系统评价。Medline,搜索Embase和Cochrane图书馆数据库，直到2024年3月。我们使用Cochrane偏差风险2.0工具（RoB2）进行随机临床试验，使用Cochrane风险非随机研究（ROBINS-I）工具进行观察性研究，评估纳入研究的质量。
结果：从确定的1242项研究中，包括15项研究，其中14项是观察性研究。总的来说,纳入了361例SSc患者，295人接受IVIG治疗。大多数研究使用2g/kgIVIG的剂量。十项研究，包括临床试验,表现出很高的偏见风险，五个有严重的风险。使用改良的Rodnan皮肤评分评估皮肤受累情况，在11项研究中，作者报告了其中9项的皮肤功效。评估肌肉受累的6项研究报告了改善。六项研究报告了胃肠道功效的数据。评估了其他领域，例如肺和关节受累以及保留类固醇的作用。最常见的不良事件是轻度的，包括头痛,腹痛,发烧,还有皮疹.
结论：在SSc患者中使用IVIG治疗可能对皮肤，肌肉，或消化表现。
