Department of Dermatology, The University of Tokyo Graduate School of Medicine, Tokyo, Japan.
Rheumatology (Oxford). 2023 Sep 1;62(9):3139-3145. doi: 10.1093/rheumatology/kead093.
SSc is an autoimmune disease characterized by excessive fibrosis in multiple organs, including the gastrointestinal (GI) tract. GI symptoms of SSc such as intestinal pseudo-obstruction (IPO) are often refractory to conventional intervention and can result in longer in-hospital stay or even increased mortality. We aimed to summarize the insights to date regarding the efficacy of IVIG against GI symptoms of SSc to unveil what we should focus on in future studies.
Herein we report the response of GI symptoms in three cases with SSc-myositis overlap who received IVIG administration. We also conducted a systematic literature review to summarize previous reports regarding the efficacy of IVIG upon the GI manifestations of SSc, according to the PRISMA 2020 guideline.
The case series demonstrated remarkable and rapid improvement of GI symptoms, including IPO, after IVIG administration. The literature review revealed that previous reports also support the efficacy and safety of IVIG against GI manifestations of SSc. However, they were all retrospective studies and lacking description of the short-term outcome after IVIG administration with objective and quantitative metrics.
IVIG seems to be a promising therapeutic option for the management of GI symptoms in SSc, including IPO. Investigators should focus more on short-term outcomes to properly assess the therapeutic benefit of IVIG, ideally using reliable quantitative measures in a multicentre randomized placebo-controlled setting.
SSc 是一种自身免疫性疾病，其特征是在多个器官中存在过度纤维化，包括胃肠道（GI）道。SSc 的 GI 症状，如假性肠梗阻（IPO），往往对常规干预措施具有抗性，并且可能导致住院时间延长，甚至死亡率增加。我们旨在总结目前关于 IVIG 对 SSc GI 症状疗效的研究进展，以揭示我们在未来研究中应关注的重点。
本文报告了三例重叠性皮肌炎的 SSc 患者接受 IVIG 治疗后 GI 症状的反应。我们还根据 PRISMA 2020 指南，对既往关于 IVIG 对 SSc GI 表现疗效的文献进行了系统综述。
该病例系列研究表明，IVIG 治疗后，包括 IPO 在内的 GI 症状迅速显著改善。文献综述表明，既往报告也支持 IVIG 对 SSc GI 表现的疗效和安全性。然而，它们均为回顾性研究，缺乏对 IVIG 治疗后短期结局的描述，也没有使用客观和定量指标。
IVIG 似乎是治疗 SSc 包括 IPO 在内的 GI 症状的一种有前途的治疗选择。研究人员应更加关注短期结局，以正确评估 IVIG 的治疗益处，理想情况下在多中心随机安慰剂对照研究中使用可靠的定量指标。
