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. 2021 Feb;6(1):37-43.
doi: 10.1177/2397198319891282. Epub 2019 Dec 20.

Gastrointestinal involvement in systemic sclerosis: Effects on morbidity and mortality and new therapeutic approaches

Affiliations

Gastrointestinal involvement in systemic sclerosis: Effects on morbidity and mortality and new therapeutic approaches

Anna-Maria Hoffmann-Vold et al. J Scleroderma Relat Disord. 2021 Feb.

Abstract

The majority of research studies in systemic sclerosis focus largely on addressing skin and cardiopulmonary manifestations. Fewer studies assess the pathogenesis and treatment of gastrointestinal tract involvement in systemic sclerosis, despite the fact that the majority of patients with systemic sclerosis have gastrointestinal manifestations and these manifestations are a leading cause of death in systemic sclerosis. The present review provides a comprehensive update on morbidity and mortality outcomes related to gastrointestinal involvement in systemic sclerosis. This review also describes conventional and emerging approaches to managing gastrointestinal symptoms in systemic sclerosis. Recent developments in systemic sclerosis-gastrointestinal research efforts have revealed promising treatment targets, including specific auto-antibodies and microbiota alterations. This review will conclude with an overview of future research directions that may improve our understanding of systemic sclerosis-gastrointestinal involvement and ultimately help to alleviate suffering from this devastating dimension of systemic sclerosis.

Keywords: Systemic sclerosis; gastrointestinal; microbiota; motility; scleroderma.

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Conflict of interest statement

Declaration of conflicting interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
The impact of organ manifestations on systemic sclerosis patients’ daily life. The gastrointestinal tract was one of the organ systems which had the greatest impact on severity assessment reported by SSc patients. The impact of organ involvement was assessed on a scale ranging from 0 (no impact) to 10 (extremely severe impact).
Figure 2.
Figure 2.
Causes of deaths in systemic sclerosis patients from the Pittsburg cohort (in blue) between 1972 and 2002 and the EUSTAR database (in red) from 2001 to 2011. The results are presented as percentage of deaths caused by gastrointestinal disease.
Figure 3.
Figure 3.
Evolution of the UCLA GIT score from week 0 to week 16 after two infusions of gut microbiota at week 0 and week 2. Three patients in the fecal microbiota transplantation (FMT) group (left panel) had apparent diarrhea at baseline, shown as values above the blue line marking clinical meaningful diarrhea, and two patients in the placebo group (right panel, FMT group). All patients treated with FMT showed a clinical meaningful effect in the diarrhea score, shown as values below the orange line marking the minimal clinical important difference measured by the UCLA GIT 2.0 score, while none of the placebo group showed an improvement.

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