Gastrointestinal Manifestations of Systemic Sclerosis

Abstract

Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibroproliferative alterations of the microvasculature leading to fibrosis and loss of function of the skin and internal organs. Gastrointestinal manifestations of SSc are the most commonly encountered complications of the disease affecting nearly 90% of the SSc population. Among these complications, the esophagus and the anorectum are the most commonly affected. However, this devastating disorder does not spare any part of the gastrointestinal tract (GIT), and includes the oral cavity, esophagus, stomach, small and large bowels as well as the liver and pancreas. In this review, we present the current understanding of the pathophysiologic mechanisms of SSc including vasculopathy, endothelial to mesenchymal transformation as well as the autoimmune pathogenetic pathways. We also discuss the clinical presentation and diagnosis of each part of the GIT affected by SSc. Finally, we highlight the latest developments in the management of this disease, addressing the severe malnutrition that affects this vulnerable patient population and ways to assess and improve the nutritional status of the patients.

Dynamics of digital ulcers in systemic sclerosis

“…The disease pathogenesis is complex, partially known. Under the action of viruses, anti-endothelial antibodies, cytotoxic T lymphocytes and endothelial cells are activated early in SSc evolution and proliferation of the innermost vessels is triggered (5). It has been observed that microvascular endothelial dysfunction has as substrate the appearance of anti-endothelial antibodies and, mainly, the presence of anti-intercellular adhesion molecule (ICAM)-1 antibodies (6).…”

Detecting gastrointestinal manifestations in patients with systemic sclerosis using anti-gAChR antibodies

“…The GI tract is the commonly involved internal organ in SSc. Vascular changes, collagen accumulation in the submucosa, and smooth muscle atrophy are histological hallmarks of SSc found in the digestive walls of patient biopsies and autopsies [16,20,21]. However, recently, the association between Abs and SSc GI damage has attracted great interest.…”

“…Systemic sclerosis (SSc), a multi-systemic disorder of the connective tissues, is characterized by widespread vascular damage and fibrosis of the skin and visceral organs [13,14] Gastrointestinal (GI) manifestations occur frequently in patients with SSc [15][16][17]. Pseudo-obstruction, malabsorption, gastroesophageal reflux disease (GERD), nausea, vomiting, constipation, and diarrhea are some of the GI complications that can occur in SSc [18][19][20][21][22].…”

Systemic Sclerosis

“…Symptoms of esophageal disease depend on dysmotility and reflux, and related to this, dysphagia, odynophagia, regurgitation, pyrosis, chronic cough, or hoarseness can occur. Stricture, Barrett's esophagus, aspiration pneumonia, and adenocarcinoma may develop as complications in these patients [27,28]. The two most common symptoms of SSc in the stomach are gastroparesis and gastric antral vascular ectasia (GAVE).…”

“…A typical striped watermelon appearance is present. Iron deficiency anemia due to GAVE may also be seen [28]. After bacterial growth due to small intestinal involvement, diarrhea and malabsorption or pseudo-obstruction due to hypomotility and dilatation may be observed [28].…”