Elsevier

La Presse Médicale

Volume 43, Issue 10, Part 2, October 2014, Pages e279-e291
La Presse Médicale

Quarterly Medical Review
Gastrointestinal involvement in systemic sclerosis

https://doi.org/10.1016/j.lpm.2014.03.029Get rights and content

Summary

Systemic sclerosis is an autoimmune chronic disease characterised by microvascular, muscular and immunologic abnormalities that lead to progressive and systemic deposition of connective tissue in the skin and internal organs. The gastrointestinal tract is often overlooked by physicians but it is the most affected organ after the skin, from the mouth to the anus. Indeed, 80% of SSc patients may present with gastrointestinal involvement. Gastrointestinal manifestations range from bloating and heartburn to dysphagia and anorectal dysfunction to severe weight loss and malabsorption. However, the gastrointestinal involvement is rarely the direct cause of death, but has great impact on quality of life and leads to several comorbidities that subsequently affect patients’ survival. Treatments, including nutritional support and prokinetics provide limited benefits and do not arrest the progressive course of the disease, but earlier detection of gastrointestinal involvement may reduce the risk of complications such as malnutrition.

Section snippets

Oral cavity

The manifestations of systemic sclerosis (SSc) at the level of the oral cavity may restrict mouth opening (microstomia) and may decrease saliva production (xerostomia), thus interfering oral intake or mastication and causing difficulty in swallowing and delayed neutralization of refluxed gastric content [10], [11], [12], [13]. Moreover, in up to 20% of patients, sicca symptoms or Sjogren's syndrome can also develop. The gingiva, taste buds and oral mucosa become brittle and atrophic, causing

Esophagus

Based on symptoms and diagnostic studies, the esophagus is the most commonly affected organ in patients with SSc [10]. Functional studies showed decreased contractile amplitude and a low resting pressure of the lower esophageal sphincter (LES) [27], [28]. The classic findings are an aperistaltic tubular esophagus and an impaired barrier function of the LES (figure 1). A large number of studies described the prevalence of esophageal motility abnormalities. In particular, the prevalence of

Stomach

Gastric involvement occurs in at least 50% of SSc patients and is associated with increased morbidity and mortality [100], [101]. The most common gastric manifestations are: delayed gastric emptying and gastric antral vascular ectasia (GAVE), also known as “watermelon stomach” [102], [103]. Gastric emptying is delayed in 10 to 75% of SSc patients, [9], [29], [104], [105], [106] also varying in relation to the diagnostic method used. Studies on the prevalence of SSc patients with GAVE are rare.

Liver

Despite SSc is a multisystem autoimmune rheumatic disease, the hepatic impairment is rare. Milbradt, in 1934, firstly described liver disease in association with scleroderma. However, in a retrospective study of post-mortem findings in scleroderma, hepatomegaly and cirrhosis were both more common in the control group than in patients with SSc [42].

Small bowel and colon

In patients with SSc, the lower GI tract is often overlooked by physicians. However, up to 50% of patients with SSc had small bowel and colonic manifestations [34], [42], [155], [156] and several studies have demonstrated alterations of intestinal motility in 80% of patients [157], [158].

Intestinal SSc is characterized by motility dysfunction and mucosal damage. Myogenic and neurogenic factors have been involved in the pathogenesis of these abnormalities [35], [159]. Progressive fibrosis that

Conclusions

Gastrointestinal manifestations of SSc are very common affecting up to 80% of patients with SSc and abnormalities can develop from the oral cavity to the anus. The most common gastrointestinal manifestations include gastroesophageal reflux disease and gastroparesis, although small bowel and colon are commonly involved as well. The liver and the lower GI tract are often overlooked by the physicians. A multidisciplinary assessment is recommended as an earlier management of these latter organs may

Disclosure of interest

the authors declare that they have no conflicts of interest concerning this article.

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