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

Abstract

Objectives Systemic sclerosis (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. Methods 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. Results 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. Conclusion 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.

Reliability, validity, and sensitivity of the Japanese version of the University of California Los Angeles scleroderma clinical trial consortium gastrointestinal tract instrument: Application to efficacy assessment of intravenous immunoglobulin administration

“…In a prior publication, we presented evidence of rapid alleviation of subjective symptoms and imaging findings of SSc-related GIT symptoms such as intestinal pseudo-obstruction and, moreover, weight recovery and weaning from total parenteral nutrition following regular monthly IVIG treatments in patients with SSc-myositis overlap. 7 Our current study reaffirmed the immediate effectiveness of IVIG, as reflected in the improvement of the GIT score. These findings underscore the utility of the GIT score as a tool for evaluating the effectiveness of IVIG in Japanese SSc patients within real-world clinical settings and, prospectively, in forthcoming clinical trials.…”

“…Furthermore, recent investigations have underscored the advantages of intravenous immunoglobulin (IVIG), one of the conventional agents being tried for SSc management characterized by its low adverse event profile, for mitigating the GIT symptoms of SSc. 7 As such, the development of clinical outcome measures that are robust, valid, and sufficiently sensitive for use in clinical trials is imperative to assess the efficacy of these groundbreaking therapies on the GIT symptoms of SSc.…”

Reliability, validity, and sensitivity of Japanese version of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument: application to efficacy assessment of intravenous immunoglobulin administration

“…In a prior publication, we presented evidence of rapid alleviation of subjective symptoms and imaging findings of SSc-related GIT symptoms such as intestinal pseudo-obstruction, and moreover, weight recovery and weaning from total parenteral nutrition following regular monthly IVIG treatments in patients with SSc-myositis overlap. [7] Our current study reaffirmed the immediate effectiveness of IVIG, as reflected in the improvement of the GIT score. These finding underscores the utility of the GIT score as a tool for evaluating the effectiveness of IVIG in Japanese SSc patients within real-world clinical settings and, prospectively, in forthcoming clinical trials.…”

“…Furthermore, we gauged the sensitivity of Japanese version of the GIT score by comparing scores before and after the administration of IVIG, which demonstrated rapid improvement of GIT symptoms of SSc in a previous study. [7] In this study, our primary objective is to establish this questionnaire as a benchmark tool for evaluating therapeutic efficacy in clinical trials involving the Japanese population.…”

Gastric dysrhythmias in patients with early systemic sclerosis: a cross-sectional study

“…There is limited and conflicting data regarding the use of immunosuppression or immunomodulation for the prevention or treatment of GI disease. Preclinical data and small observational studies suggest that immunomodulation with IVIG may benefit a subset of SSc patients with GI involvement, especially those with pseudo-obstruction [ 33 , 34 ].…”