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Table 1. Therapeutic Management of Gastrointestinal Manifestations of Systemic Sclerosis.  


Management of Gastrointestinal Manifestations in Systemic Sclerosis (Scleroderma)

  • Authors: Ghaith Noaiseh, MD; Sophia Li, MD; Chris T. Derk, MD, MSc
  • CME Released: 11/26/2012
  • Valid for credit through: 11/26/2013
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Target Audience and Goal Statement

This activity is intended for gastroenterologists, rheumatologists, and other clinicians caring for patients with GI involvement of SSc.

The goal of this activity is to review presentation and management of GI involvement in patients with SSc.

Upon completion of this activity, participants will be able to:

  1. Describe esophageal and gastric involvement in SSc, based on a review
  2. Describe small intestine involvement and malabsorption in SSc, based on a review
  3. Describe large intestine involvement in SSc, based on a review


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  • Ghaith Noaiseh, MD

    Division of Rheumatology, University of Pennsylvania, Philadelphia


    Disclosure: Ghaith Noaiseh, MD, has disclosed no relevant financial relationships.

  • Sophia Li, MD

    Division of Rheumatology, University of Pennsylvania, Philadelphia


    Disclosure: Sophia Li, MD, has disclosed no relevant financial relationships.

  • Chris T. Derk, MD, MSc

    Division of Rheumatology, University of Pennsylvania, Philadelphia


    Disclosure: Chris T. Derk, MD, MSc, has disclosed no relevant financial relationships.


  • Elisa Manzotti

    Publisher, Future Science Group, London, United Kingdom


    Disclosure: Elisa Manzotti has disclosed no relevant financial relationships.

CME Author

  • Laurie Barclay, MD

    Freelance writer and reviewer, Medscape, LLC


    Disclosure: Laurie Barclay, MD, has disclosed no relevant financial relationships.

CME Reviewer

  • Nafeez Zawahir, MD

    CME Clinical Director, Medscape, LLC


    Disclosure: Nafeez Zawahir, MD, has disclosed no relevant financial relationships.

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Management of Gastrointestinal Manifestations in Systemic Sclerosis (Scleroderma): Esophageal Involvement


Esophageal Involvement

The esophagus is the most commonly involved organ of the GI tract in SSc,[4] and in the early stages of SSc, esophageal manometry studies show low amplitude peristaltic contractures, a low lower esophageal sphincter (LES) pressure and failure of the LES to relax.[11] In mid-stage SSc, the decreased peristaltic amplitude progresses into absent peristalsis of the lower two-thirds of the esophagus,[11] and myoelectrical activity between swallows are absent.[17] In late stages, LES is almost completely absent[11] and the function of the upper third of the esophagus may also be involved, which may lead to even worse consequences due to loss of the last barrier that prevents aspiration.[11]

Gastroesophageal reflux disease (GERD) is a major clinical finding in scleroderma patients. The reflux of gastric content into the esophagus and the resultant mucosal injury is not only facilitated by a lower LES pressure, but also by the inability of the lower esophagus muscle peristalsis to effectively clear the esophageal content.[17,18] The latter mechanism may be the main contributor to esophageal injury from acid exposure in SSc patients.[18]

Patients with GERD complain of dysphagia, odynophagia, heartburn and regurgitation. Prolonged GERD may lead to erosive esophagitis, esophageal ulceration and stricture of the distal esophagus, and in the worse cases Barrett’s esophagus that can lead to esophageal cancer.[4] Not all patients with GERD are symptomatic. Endoscopic studies showed that a significant proportion of patients with SSc have evidence of esophagitis in the absence of any symptoms,[19] which highlights the importance of early screening for GERD in this population.

The management of GERD in systemic sclerosis incorporates pharmacological therapy and lifestyle modifications.[20] Patients are advised to elevate the head of the bed, avoid going to bed for 3–4 h following a meal, lose weight, stop smoking and minimize alcohol use. Patients are also encouraged to avoid using beverages or medications that can further lower the LES pressure such as caffeine-containing drinks, chocolate, calcium channel blockers and nitrates. Patients should be given strict instructions when prescribed medications that can cause esophageal irritation (bisphosphonates, tetracyclines, iron and NSAIDs), such as to avoid lying down or bending for 1–2 h after use and to take them with a full glass of water. In some cases, these medications should be avoided altogether.

Proton pump inhibitors (PPIs) play a major role in the management of GERD in scleroderma patients. Omeprazole has been shown to heal esophagitis[21] and may even reverse esophageal fibrosis.[22] The use of a higher than standard dose has been shown to control symptoms in most patients without major side effects.[21] In a recent randomized, placebo-controlled trial involving 24 patients, lansoprazole, at a standard dose of 30 mg daily was effective in controlling the symptoms of GERD in SSc patients in the first 6 months of therapy, but not after 1 year. The authors concluded that inadequate response may be the result of inadequate dosing.[23] Starting PPIs in all SSc patients early in the course of the disease should be considered.[10,24] Physicians should explain to patients that PPI use is lifelong.[20]

In the PPI era, H2-blockers may still have some role when used in conjunction with PPI, particularly if nocturnal symptoms are present despite maximal PPI therapy.[20] Prokinetic agents, such as cisapride, work by increasing the LES pressure, improving peristalsis and esophageal clearance, and by increasing gastric emptying, thus improving symptoms.[25,26] There is a paucity in the literature about the exact role of prokinetic agents use in conjunction with acid suppressive therapy for the esophageal manifestations of SSc, although some authors advise this combination when concomitant gastroparesis is present.[20]

More recent work has focused on GABA[B] agonists, and metabotropic glutamate receptor antagonists (mGluR), both of which have been shown to increase basal LES pressure. Baclofen, the prototypic GABA(B) agonist was used at a dose of 40 mg, 90 min before a meal and it inhibited reflux episodes in up to 40–60% of healthy volunteers,[27] although CNS side effects may limit its use. A proof-of-concept study has recently also looked at mGlu R modulator ADX10059. During 24 h of esophageal pH monitoring, 250 mg of baclofen given three-times daily reduced esophageal acid exposure from 7.2 to 3.6 % of the total monitoring time in healthy volunteers.[28] Neither of these agents has been looked at in SSc-GERD.

The incidence of Candida esophagitis is increased in SSc patients with GERD, particularly those on chronic acid suppressive therapy,[11,29] and although therapy for 2–4 weeks with fluconazole is effective in eradicating the infection, it recurred in almost all of the patients.[29]

In general, surgical antireflux therapy should be approached with caution in scleroderma patients with severe GERD since the procedure may itself further worsen the emptying capacity of an already-damaged distal esophagus.[20] Nevertheless, fundoplication has been shown to reduce GERD in SSc patients,[30] while esophageal strictures are managed by endoscopic dilatation of the involved area.

Barrett’s Esophagus

Chronic GERD can lead to metaplasia of the lower esophageal mucosa, whereby normal squamous epithelium is replaced by columnar and goblet cells, and this is characterized as Barrett’s esophagus (BE). In total, 5–15% of patients develop BE due to chronic GERD regardless of etiology.[31,32] One report estimated that 12.7% of SSc patients with GERD developed BE over a 2-year follow-up while on a PPI.[33] BE can frequently lead to dysplasia and these patients are at increased risk for adenocarcinoma,[34] while specific recommendations for the best endoscopic follow-up strategy in SSc-related GERD patients needs to be assessed in future studies, patients with GERD and BE should undergo endoscopy every 2–3 years. If low-grade dysplasia is diagnosed, a yearly endoscopy is recommended and every 3 months if high-grade dysplasia is present.[32]

Lung Involvement Due to Esophageal Dysfunction

A new distinct pattern of interstitial lung disease (ILD) has been described in SSc patients called centrilobular fibrosis (CLO)[35] and has been linked to GERD. This is a pathologically different pattern than other ILD patterns described in SSc patients (nonspecific interstitial pneumonia and usual interstitial pneumonia), and thus aggressive management of GERD may result in better pulmonary outcome.[10,36]

A recent study using pH impedance monitoring in SSc patients off PPI therapy compared acid reflux in patients who had ILD on their high-resolution CT of the chest versus patients who did not have ILD, and they showed that patients with ILD were more likely to have acid and nonacid reflux episodes and a higher number of reflux episodes reaching the proximal esophagus.[37] In another study, six patients with SSc-ILD possibly related to GERD who were treated with anti-reflux therapy showed stability after 12 months.[38]