Rapid improvement of systemic sclerosis-associated intestinal pseudo-obstruction with intravenous immunoglobulin administration.

Rapid improvement of systemic sclerosis-associated intestinal pseudo-obstruction with intravenous immunoglobulin administration.
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DOI:
10.1093/rheumatology/kead093
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发表时间:
2023-09-01
期刊:
影响因子:
5.5
通讯作者:
Sato, Shinichi
Sato, Shinichi
中科院分区:
医学1区
文献类型:
--
作者:
Matsuda, Kazuki M.;Yoshizaki, Ayumi;Kuzumi, Ai;Toyama, Satoshi;Awaji, Kentaro;Miyake, Tomomi;Sato, Shinichi

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SSc是一种自身免疫性疾病,其特征在于包括胃肠道(GI)在内的多个器官中的过度纤维化。SSc的胃肠道症状,如假性肠梗阻(IPO),通常是传统干预难治性的,并可能导致住院时间更长,甚至死亡率增加。我们的目的是总结迄今为止关于IVIG对SSc胃肠道症状的疗效的见解,以揭示我们在未来研究中应该关注的问题。在此,我们报告了三例接受IVIG治疗的SSc-肌炎重叠病例的胃肠道症状反应。我们还根据PRISMA 2020指南进行了系统的文献综述,总结了先前关于IVIG对SSc GI表现的疗效的报告。该病例系列显示IVIG给药后GI症状(包括IPO)显著且快速改善。文献综述显示,先前的报告也支持IVIG对SSc GI表现的有效性和安全性。然而,它们都是回顾性研究,缺乏对IVIG给药后短期结局的客观和定量指标描述。IVIG似乎是一种有前途的治疗选择,用于管理SSc中的GI症状,包括IPO。研究者应该更多地关注短期结果,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环境中使用可靠的定量测量。
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.
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