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.

Keywords: Deglutition; Deglutition disorders; Esophageal motility disorders; Esophagitis; Gastroesophageal reflux; Systemic sclerosis.

Figure 1

The types of scleroderma and features of each disease. Scleroderma can be broken down into localized scleroderma or systemic sclerosis. Systemic sclerosis can be further broken down into limited and diffuse types. GI: Gastrointestinal.

Figure 2

Simplified pathophysiology of systemic sclerosis. SSc: Systemic sclerosis; Th2: Type 2 T helper; Th1: Type 1 T helper; TGF-beta: Transforming growth factor beta; TXA2: Thromboxane A2; ET-1: Endothelin 1; VEGF: Vascular endothelial growth factor; IL: Interleukin.

Figure 3

Disruptions of normal swallowing physiology due to disease processes of scleroderma.

Figure 4

Normal manometry findings showing peristalsis during swallowing and lower esophageal sphincter tone compared to manometry findings in scleroderma showing an absence of peristalsis and lower esophageal sphincter hypotension. LES: Lower esophageal sphincter; UES: Upper esophageal sphincter.

Figure 5

Disturbances of defense mechanisms against reflux in scleroderma. LES: Lower esophageal sphincter.

Figure 6

Twenty-four hour impedance pH monitoring normal findings compared to findings in scleroderma. In normal esophagus, there are no episodes of acid reflux, as indicated by no drops of pH below 4 in the esophagus. In scleroderma esophagus, there is prolonged acid exposure in the distal esophagus.