Abstract

Gastrointestinal tract (GIT) involvement is the most common internal organ manifestation and is present in up to 90% of patients with systemic sclerosis (SSc). Clinical manifestations can differ according to the part of the GIT affected, disease course and symptoms. A majority of the symptoms are caused by GIT dysmotility. Up to 8% of SSc patients develop several GIT symptoms, which increase the mortality. Although GIT involvement is rarely the direct cause of death, it can lead to several comorbidities including malnutrition and negative alterations of body composition. These factors have a negative impact on quality of life and increase the mortality. To date, the treatment is rather symptomatic. The pathogenesis of GIT involvement in SSc still remains to be clarified to improve the treatment approaches including intravenous immunoglobulins and microRNA interventions.

Highlights

  • Systemic sclerosis (SSc), characterised by autoimmune inflammation, vasculopathy and fibrotic tissue deposition as the main pathophysiological features, can affect any organ system

  • Up to 90% of SSc patients are affected by some degree of Gastrointestinal tract (GIT) fibrosis, with no difference in frequency in limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis subsets

  • Oesophageal dysfunction appears to be the most common GIT manifestation in SSc affecting up to 90% SSc patients with higher prevalence and tendency to deteriorate over time in diffuse cutaneous systemic sclerosis (dcSSc) compared to limited cutaneous systemic sclerosis (lcSSc) [42, 43]

Read more

Summary

Introduction

Systemic sclerosis (SSc), characterised by autoimmune inflammation, vasculopathy and fibrotic tissue deposition as the main pathophysiological features, can affect any organ system. The pathophysiology of GIT involvement in general corresponds to the skin and other organ involvement in SSc, with the main characteristic pathologic features: vascular abnormalities, immune cell infiltration in tissue, autoantibodies and typical extensive deposition of collagen fibres. This process leads to specific early myenteric neural dysfunction caused by autoantibodies and collagen deposition, vasculopathy in myointimal layer mainly in capillaries preceding the muscle changes, smooth muscle cell infiltration with mononuclear cells with consequent atrophy and fibrosis of enteric connective tissue [1, 6]. Up to 8% of SSc patients can develop severe GIT symptoms, which lead to increased mortality with only 15% survival at 9 years [5]

Pathophysiology
Clinical manifestations
Oral cavity and pharynx
Oesophagus
Stomach
Small intestine
Large intestine
Liver and pancreas
Malnutrition
Alterations of body composition
Diagnostic tools
Patient-reported outcomes
Current therapeutic options
Therapy of malnutrition
Future therapeutic prospects
Findings
Conclusion
Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call