Gastrointestinal Involvement in Systemic Sclerosis

Diagnosis and Management

Zsuzsanna H. McMahan; Laura K. Hummers


Curr Opin Rheumatol. 2018;30(6):533-540. 

In This Article



Esophageal disease in SSc is the most common gastrointestinal complication, as it affects up to 90% of patients. It is often one of the earliest features of SSc, and may present with symptoms of dysphagia, heartburn, and regurgitation. An international gastroesophageal reflux disease (GERD) working group recently published a consensus statement, incorporating published data with expert consensus opinion.[8] This working group suggests that a diagnosis of GERD is initially dependent on response to an empiric trial of antisecretory therapy. When symptoms persist, despite initial therapy, and/or if alarm symptoms are present (e.g. unintentional weight loss, anemia), then esophageal motor physiology and reflux burden may be investigated through high-resolution esophageal manometry and ambulatory pH monitoring, respectively.[8] These additional data can more specifically define the clinical problem and direct patient care towards targeted therapeutic interventions.

Esophageal manometry is the gold standard for diagnosing esophageal dysmotility in SSc.[8] Several new studies have sought to define the distribution of manometric findings in SSc. Manometric findings in SSc are heterogeneous, with recent studies suggesting that absent contractility is the most frequent finding (56%), followed by normal motility in 26%, and ineffective esophageal motility in 10%.[9] The characteristic esophageal findings in SSc of hypotensive lower esophageal sphincter and absent contractility coexist in approximately 33% of patients.[9,10] Significant overlap between the presence of esophageal involvement (defined by high-resolution manometry) and anorectal dysfunction (defined by anorectal manometry) was also recently reported, suggesting that there may be a mechanistic link between the esophageal and anorectal complications in SSc.[11] Others sought to determine whether high-resolution manometry correlates with upper gastroesophageal reflux disease symptoms,[12] and found that a negative correlation between reflux scores from the UCLA GIT 2.0 questionnaires (higher = more severe), suggesting that esophageal symptoms may serve as a good surrogate for objective esophageal data in SSc.


Gastroparesis is reported in 38–50% of SSc patients. Common presenting symptoms include early satiety, bloating, and regurgitation, though a strong association between symptoms and objective motility testing has not yet been demonstrated even in the general population. Stanford investigators demonstrated that non-SSc patients with symptoms suggestive of a functional dyspepsia and/or gastroparesis (such as postprandial distress) also had concomitant esophageal motility abnormalities.[13] Seventy-two percentage (44/61) of these symptomatic patients demonstrated evidence of gastroparesis by scintigraphy. Concomitant esophageal motility disorders were more common among patients with evidence of gastroparesis (68%) than among patients without evidence of gastroparesis (42%). Importantly, symptoms of heartburn, regurgitation, bloating, nausea, vomiting, dysphagia, and belching could not distinguish between patients with and without gastroparesis, though weight loss was more prevalent and severe in the gastroparesis group. This suggests that symptoms alone are not effective in distinguishing between the presence and absence of gastroparesis in SSc.

Small Intestine

The small intestine is affected in 12–55% of SSc patients.[14–16] Small intestinal involvement may present with symptoms of small intestinal dysmotility (e.g. distention, bloating), small intestinal bacterial overgrowth (SIBO), or both. Though the etiopathogenesis of SIBO is poorly understood, investigators recently determined that non-SSc patients with SIBO have significantly lower ileocecal junction pressure, prolonged small bowel transit time, and a higher pH as compared to those without SIBO, and it was thought that these abnormalities may play a role in SIBO pathogenesis.[17] Investigating these measures in SSc patients may provide insight into SIBO pathogenesis in SSc.

Colon and Anorectum

Constipation and fecal incontinence are reported in 50% or more of SSc patients.[18,19] A French study recently examined the prevalence of fecal incontinence in 77 SSc patients and identified risk factors associated with this complication. Findings from this cohort were compared with a historical cohort from the general population in the Rhone-Alpes region of France.[20] They found that 38% of SSc patients, and only 6% of patients in the general population had fecal incontinence. Clinical associations between SSc-related fecal incontinence and longer disease duration, loose stools, SIBO, and constipation, were also reported.[20,21] These findings suggest that control of lower bowel symptoms may improve fecal incontinence in SSc.