Dysphagia, reflux and related sequelae due to altered physiology in scleroderma

Abstract

Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical manifestation of systemic sclerosis and is strongly related to esophageal dysmotility. However, there are multiple other contributing factors in each step in the physiology of swallowing that may contribute to development of severe dysphagia. The oral phase of swallowing may be disrupted by poor mastication due to microstomia and poor dentition, as well as by xerostomia. In the pharyngeal phase of swallowing, pharyngeal muscle weakness due to concurrent myositis or cricopharyngeal muscle tightening due to acid reflux can cause disturbance. The esophageal phase of swallowing is most commonly disturbed by decreased peristalsis and esophageal dysmotility. However, it can also be affected by obstruction from chronic reflux changes, pill-induced esophagitis, or Candida esophagitis. Other contributing factors to dysphagia include difficulties in food preparation and gastroparesis. Understanding the anatomy and physiology of swallowing and evaluating systemic sclerosis patients presenting with dysphagia for disturbances in each step can allow for development of better treatment plans to improve dysphagia and overall quality of life.

Gastrointestinal involvement in systemic sclerosis: pathogenesis, assessment and treatment

“…Up to 90% of patients with SSc have symptoms of upper gastrointestinal disease [33,34] Patients may present with symptoms of laryngo-esophageal [35][36][37][38] or gastroesophageal reflux disease (GERD)…”

Esophageal Dysphagia in Children: State of the Art and Proposal for a Symptom-Based Diagnostic Approach

“…Primary or acquired immunodeficiencies as well as prolonged antibiotic and steroid treatment favor the development of IE (34,39). Immune-mediated disorders, such as systemic sclerosis (40) and Crohn's disease can rarely involve the esophagus causing ED (41). Ongoing treatment with tablets/capsules should raise the possibility of pill-induced esophagitis especially when there is a close temporal relation (<10 days) with the use of NSAIDs, antibiotics (e.g., doxycycline, amoxicillin), L-arginine and iron, and the drug has been taken with little water at bedtime (42,43).…”

Twitter debate: controversies in dysphagia

“…While there are a number of possible causes of dysphagia in systemic sclerosis (table 1), oesophageal involvement, resulting in dysphagia, is a common presentation in systemic sclerosis, affecting over 90% of patients, and may be the first manifestation of the disease. Treatment options will be targeted at the cause of dysphagia 18…”