Digestive Diseases and Sciences

, Volume 63, Issue 4, pp 834–844 | Cite as

Intestinal Involvement in Systemic Sclerosis: A Clinical Review

  • Lazaros I. SakkasEmail author
  • Theodora Simopoulou
  • Dimitrios Daoussis
  • Stamatis-Nick Liossis
  • Spyros Potamianos


Systemic sclerosis (SSc) is a chronic systemic disease characterized by microvasculopathy, autoantibodies, and extensive fibrosis. Intestinal involvement is frequent in SSc and represents a significant cause of morbidity. The pathogenesis of intestinal involvement includes vascular damage, nerve dysfunction, smooth muscle atrophy, and fibrosis, causing hypomotility, which leads to small intestinal bacterial overgrowth (SIBO), malabsorption, malnutrition, diarrhea, pseudo-obstruction, constipation, pneumatosis intestinalis, and fecal incontinence. Manifestations are often troublesome and reduce quality of life and life expectancy. Assessment of intestinal involvement includes screening for small intestine hypomotility, malnutrition, SIBO, and anorectal dysfunction. Current management of intestinal manifestations is largely inadequate. Patients with diarrhea are managed with low-fat diet, medium-chain triglycerides, avoidance of lactulose and fructose, and control of bacterial overgrowth with antibiotics for SIBO. In diarrhea/malabsorption, bile acid sequestrant and pancreatic enzyme supplementation may help, and nutritional support is needed. General measures are applied for constipation, and intestine rest plus antibiotics for pseudo-obstruction. Fecal incontinence is managed with measures for associated SIBO, or constipation, and with behavioral therapies. Pneumatosis intestinalis is usually an incidental finding that does not require any specific treatment. Immunomoduation should be considered early in intestinal involvement. Multidisciplinary approach of intestinal manifestations in SSc by gastroenterologists and rheumatologists is required for optimum management.


Bacterial overgrowth Malnutrition Pseudo-obstruction Systemic sclerosis 


Compliance with ethical standards

Conflict of Interest

The authors declare no conflicts of interest.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Lazaros I. Sakkas
    • 1
    Email author
  • Theodora Simopoulou
    • 1
  • Dimitrios Daoussis
    • 2
  • Stamatis-Nick Liossis
    • 2
  • Spyros Potamianos
    • 3
  1. 1.Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health SciencesUniversity of ThessalyLarissaGreece
  2. 2.Division of Rheumatology, Department of Medicine, Faculty of Medicine, School of Health SciencesUniversity of PatrasPatrasGreece
  3. 3.Department of Gastroenterology, Faculty of Medicine, School of Health SciencesUniversity of ThessalyLarissaGreece

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