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

Table 1 Features of various diagnostic studies in systemic sclerosis diagnosis

Diagnostic study	Role in SSc
Esophagogastroduodenoscopy	Evaluates for esophageal causes of dysphagia[4]
	Shows reflux-related complications: erosive esophagitis, strictures, Barrett’s esophagus, esophageal adenocarcinoma[4]
	Reveals esophageal findings in asymptomatic patients[4]
Esophageal manometry	Detects esophageal dysmotility, even in early stages of SSc[4]
	Shows decreased lower esophageal sphincter pressure and absent peristalsis in distal two-thirds of esophagus[4]
Pharyngeal manometry	Evaluates for oropharyngeal dysphagia by assessing upper esophageal sphincter relaxation and pharyngeal propulsion[38]
Esophageal pH monitoring (with or without impedance)	Gold standard for gastroesophageal reflux detection[4]
	Used for patients with resistant reflux[4]
Videofluorography swallow study of esophagus	Shows esophageal dysmotility with decreased peristalsis in distal 2/3 of esophagus[13]
	Shows decrease of lower esophageal sphincter pressure[13]
	Shows dilated lumen of esophagus[13]
CT chest	Shows esophageal dilation[13]

SSc: Systemic sclerosis; CT: Computed tomography.

Table 2 Scoring system of gastrointestinal symptoms based on UCLA Scleroderma Clinical Trial Consortium GIT 2.0[14]

Scales	None-to-mild symptoms	Moderate symptoms	Severe-to-very severe symptoms
Reflux
Distension/bloating
Diarrhea
Constipation
Fecal soilage
Emotional well being
Social functioning
Total GIT score

Table 3 Treatment options for the various disease processes that contribute to the development of dysphagia in systemic sclerosis

Disease process	Therapeutic plan
Xerostomia	Drinking water more frequently[2]
	Using artificial saliva as needed[2]
	Using special toothpastes and mouthwashes[2]
	Avoiding medications that exacerbate dry mouth[2]
Microstomia	Performing exercises and massages to stretch the mouth[2]
Dental decay	Planning regular follow-up with experienced dentist[2]
Concurrent myositis	Frequent screening for myositis in patients with SSc and suggestive symptoms[37]
	Treating concurrent myositis with immunomodulatory therapy and interventional procedures[37]
Esophageal Dysmotility	Lifestyle management (taking smaller bites, chewing food thoroughly, drinking adequate water with food)[16]
GERD	Medications (PPIs, H2RAs)[2,52]
	Dietary modifications (avoiding acidic foods)[52]
	Lifestyle modifications (avoiding meals before bedtime, elevating the head of the bed while sleeping)[2,57]
Candida esophagitis	Screening for fungal esophagitis in patients with SSc and suggestive symptoms[73]
	Prompt antifungal treatment[73]
Pill esophagitis	Avoiding medications at high risk of causing esophagitis[79]
	Screening for esophagitis in SSc patients taking culprit medications with suggestive symptoms[79]
Gastroparesis	Medications (prokinetic agents)[2]
	Dietary modifications of small frequent meals with fiber[2]

SSc: Systemic sclerosis; PPIs: Proton pump inhibitors; H2RAs: H2-receptor antagonists; GERD: Gastroesophageal reflux disease.