Quarterly Medical ReviewGastrointestinal involvement in systemic sclerosis
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.
References (179)
- et al.
Analysis of the oral manifestations of systemic sclerosis (scleroderma)
Oral Surg Oral Med Oral Pathol
(1988) - et al.
Scleroderma: oral manifestations
Oral Surg Oral Med Oral Pathol
(1981) - et al.
Oral radiographic changes in patients with progressive systemic sclerosis (scleroderma)
J Am Dent Assoc
(1977) Unilateral osteolysis of the mandibular angle and coronoid process in scleroderma
Int J Oral Maxillofac Surg
(1988)- et al.
Severe gastrointestinal involvement in systemic sclerosis: report of five cases and review of the literature
Semin Arthritis Rheum
(2005) - et al.
Comparative esophageal and anorectal motility in scleroderma
Gastroenterology
(1985) - et al.
Clinical and upper gastrointestinal motility features in systemic sclerosis and related disorders
Am J Gastroenterol
(1998) The gastrointestinal manifestations of scleroderma: pathogenesis and management
Gastroenterology
(1980)- et al.
Myenteric neuronal antibodies in scleroderma: passive transfer evokes alterations in intestinal myoelectric activity in a rat model
J Lab Clin Med
(1999) - et al.
Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls
Am J Med
(1969)