The role of surgery in esophageal involvement in systemic sclerosis: A systematic literature review

Background

Gastroesophageal reflux disease (GERD) is one of the earliest clinical manifestations of systemic sclerosis (SSc). Proton pump inhibitors are ineffective in controlling symptoms in up to 40 % of cases, and their chronic use (often) at high-doses and long-term safety are a concern. For this reason, surgery has been proposed as an alternative management strategy for refractory GERD in SSc patients. However, consensus on the optimal surgical procedure, timing, and ideal patient population, is still lacking.

Objectives

To evaluate and compare the safety, efficacy, indications, timing and feasibility of different surgical approaches to fundoplication (FP) for treating SSc-related refractory GERD.

Methods

Four research questions based on the PICO framework were developed to guide the systematic literature review (SLR) which was completed 01 September 2024. The search was performed across different databases including PubMed, MEDLINE (OVID), EMBASE, Cochrane Library, Web of Science, Google Scholar, Emcare and Academic Search Premier. References were independently screened by two reviewers who also independently assessed the full text of eligible articles and extracted data. Due to the heterogeneity of retrieved studies, narrative summaries were used to present the data.

Results

Of the 986 retrieved papers, 23 were eligible for inclusion. In these studies, 184 SSc patients had an anti-reflux surgical procedure and were included in the analysis. Most of the studies were conducted in surgical settings and relevant rheumatological data were largely missing. Refractory GERD symptoms were the most common indication for surgery, with post-operative dysphagia being the most frequent complication. In 14 studies, FP was effective in reducing the GERD symptoms, 1 study had indeterminate findings while in 4 studies, unfavourable results for FP were reported. All the included studies reported a low mortality and morbidity rate related to surgery.

Conclusions

Our data demonstrate that the surgical management of refractory GERD remains a challenge due to the limited evidence, which is largely of poor quality. However, the safety and beneficial effect of FP obtained in the majority of SSc patients, suggests that it may be a beneficial intervention in some patients. The definition of minimal requirements to perform surgical studies on refractory GERD, identifying a specific SSc subset of patients that would most likely benefit from FP, and determining the optimal timing for surgery are warranted.