Title: Systemic Scleroderma - NORD (National Organization for Rare Disorders)
Headings: Rare Disease Database
Rare Disease Database
Systemic Scleroderma
Synonyms of Systemic Scleroderma
Subdivisions of Systemic Scleroderma
General Discussion
Signs & Symptoms
Causes
Affected Populations
Diagnosis
Standard Therapies
Treatment & Management
Investigational Therapies
NORD Member Organizations
Other Organizations
References
Years Published

Content: The two main symptoms of ILD are progressive dyspnea and dry cough. PAH and ILD are progressive conditions and can eventually become life-threatening. Taken together, they account for about half of all deaths related to systemic scleroderma. Gastrointestinal tract
The most commonly involved part of the gastrointestinal tract in systemic scleroderma is the esophagus. In affected individuals, the esophagus is unable to contract efficiently (esophageal dismotility) to allow food to reach the stomach. In addition, the lower esophageal sphincter (LES), which is a circular muscle between the esophagus and the stomach, is not able to close properly. The combination of esophageal dismotility and LES incompetence can lead to heartburn due to acid reflux (a condition known as gastroesophageal reflux disease; GERD), choking on food, and difficulty swallowing (dysphagia). Fibrosis of the rest of the gastrointestinal can prevent food from moving through properly (pseudo-obstruction), which can promote bacterial growth and lead to pain, bloating, constipation, diarrhea, and fecal incontinence. Gastrointestinal bleeding also occurs in some patients due to dilation of veins in the stomach (gastric venous ectasias).