Gastrointestinal Manifestations of Systemic Sclerosis

Isabel M McFarlane, Manjeet S Bhamra, Alexandra Kreps, Sadat Iqbal, Firas Al-Ani, Carla Saladini-Aponte, Christon Grant, Soberjot Singh, Khalid Awwal, Kristaq Koci, Yair Saperstein, Fray M Arroyo-Mercado, Derek B Laskar, Purna Atluri, Isabel M McFarlane, Manjeet S Bhamra, Alexandra Kreps, Sadat Iqbal, Firas Al-Ani, Carla Saladini-Aponte, Christon Grant, Soberjot Singh, Khalid Awwal, Kristaq Koci, Yair Saperstein, Fray M Arroyo-Mercado, Derek B Laskar, Purna Atluri

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.

Keywords: Diagnosis; Esophagus; Gastrointestinal; Interstitial lung disease; Management; Pathogenesis; Scleroderma; Systemic sclerosis.

Figures

Figure 1
Figure 1
Pathogenesis of Systemic Sclerosis. ET: Endothelin; IL: Interleukin; ROS: Reactive Oxygen Species; PAH: Pulmonary Arterial Hypertension; CD: Cluster of Differentiation; ADCC; Antibody-Dependent Cellular Cytotoxicity; Anti-EC: Anti- Endothelial Cell; IP: Interferon-gamma Induced Protein; LPA: Lysophosphatidic Acid; MMP: Matrix Metalloproteinases; TGF-β Tissue Growth Factor β; MCP Monocyte Chemoattractant Protein.
Figure 2
Figure 2
Pathogenesis of Gastrointestinal Involvement in Systemic Sclerosis.
Figure 3
Figure 3
CT Abdomen with oral contrast in a patient with SSc demonstrates profound dilation of the distal lower third of the esophagus with 30 mm dilation and layer of intraluminal contrast.
Figure 4
Figure 4
Summary of pathogenesis of ILD and relationship to GERD.
Figure 5
Figure 5
Low-power view shows columnar-lined epithelium, gastric pits, and underlying mucous glands with scattered chronic inflammatory cells within the underlying lamina classically seen with Barrett’s changes.
Figure 6
Figure 6
Loss of haustra due to weakened wall integrity noted in the ascending limb of the large bowel.
Figure 7
Figure 7
Colon and anorectal involvement in Systemic Sclerosis.

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