What is the recommended diagnostic work‑up and management for esophageal dysmotility and gastro‑esophageal reflux in a patient with systemic sclerosis?

Esophageal Abnormalities in Systemic Sclerosis: Diagnostic Work-Up and Management

Diagnostic Evaluation

All patients with systemic sclerosis should undergo upper gastrointestinal endoscopy as the first-line diagnostic test to identify esophagitis, strictures, Barrett's esophagus, and other complications of gastroesophageal reflux disease. 1, 2

Essential Diagnostic Tests

• Upper endoscopy reveals erosive esophagitis in approximately 85% of patients with systemic sclerosis and identifies complications including hiatal hernia, strictures, and Barrett's esophagus 3, 4
• High-resolution manometry is the preferred method for characterizing esophageal dysmotility, showing absent contractility in 60% of patients, ineffective esophageal motility in 18%, and normal findings in only 19% 1, 4
• 24-hour pH monitoring demonstrates pathologic acid exposure in 83-91% of systemic sclerosis patients and should be performed when diagnosis remains unclear or symptoms persist despite therapy 5, 2

Classic Manometric Pattern

The hallmark finding is absent or ineffective peristalsis of the distal esophagus combined with hypotensive lower esophageal sphincter, present in 55-73% of patients 1, 4. This pattern results from smooth muscle atrophy and fibrosis, leading to severe motility disturbance 2.

Management Strategy

First-Line Pharmacologic Therapy

Proton pump inhibitors are the cornerstone of treatment and should be used at high doses, often exceeding maximum approved doses, to prevent esophageal ulcers and strictures. 6

• PPIs must be initiated immediately as they prevent progression to Barrett's esophagus and stricture formation 6
• High-dose PPI therapy is necessary because standard doses are frequently insufficient in systemic sclerosis 6

Adjunctive Prokinetic Therapy

Add prokinetic agents when symptoms persist despite adequate PPI therapy, selecting the agent based on symptom pattern and disease stage. 7

• Domperidone is the preferred first-line prokinetic, showing 86.8% response rate when added to ongoing PPI therapy in a randomized trial of 148 patients 7
• Prucalopride should be used for patients with both upper and lower gastrointestinal symptoms, particularly when constipation is prominent 7
• Buspirone is reserved for refractory cases with lower esophageal sphincter dysfunction, as it increases sphincter pressure more effectively than domperidone 7, 1
• Avoid metoclopramide for long-term use due to significant adverse effects including tardive dyskinesia 7

Critical caveat: Prokinetic agents are only effective in early disease stages when gastrointestinal musculature remains intact; they become ineffective once fibrosis predominates 7.

Aggressive Anti-Reflux Lifestyle Modifications

Implement rigorous lifestyle measures as these are essential adjuncts to pharmacotherapy and directly reduce aspiration risk that worsens interstitial lung disease. 6

• Elevate the head of the bed to prevent nocturnal reflux 6
• Prohibit food intake after dinner to minimize overnight acid exposure 6
• Ensure thorough chewing, slow eating, and drinking water with meals to compensate for impaired esophageal clearance 6
• Avoid foods that trigger reflux or worsen dysphagia 6

Management of Dysphagia and Strictures

• Esophageal dilations should be performed when strictures develop, as these are common complications in patients with severe esophageal involvement 6, 3
• H2 antagonists, sucralfate, and antacids can be added as supplementary therapy when PPI monotherapy is insufficient 6

Surgical Intervention

Consider fundoplication surgery only for severe reflux with aspiration symptoms, but recognize that Roux-en-Y gastric bypass may provide superior outcomes in patients with significant esophageal dysmotility. 6, 3

• Fundoplication has a 50% symptom improvement rate in systemic sclerosis patients, significantly lower than in the general population 3
• Roux-en-Y gastric bypass achieved 100% symptom improvement in a comparative study and may be the preferred surgical option when esophageal dysmotility is severe 3
• Surgery should be reserved for resistant cases after exhausting medical management 2

Critical Link to Pulmonary Disease

Aggressive treatment of gastroesophageal reflux disease is mandatory because severe esophageal involvement—particularly symptoms of aspiration, nocturnal reflux, and significant dysphagia—directly increases the risk of interstitial lung disease progression. 6

• Aspiration from reflux worsens interstitial lung disease and increases mortality 6
• Patients with severe esophageal symptoms require more intensive monitoring for pulmonary complications 6

Monitoring for Small Intestinal Bacterial Overgrowth

Use intermittent or rotating antibiotics to treat symptomatic small intestinal bacterial overgrowth, which commonly develops secondary to chronic intestinal dysmotility. 6, 7

• Small intestinal bacterial overgrowth is a frequent complication of prolonged esophageal and intestinal dysmotility 6
• Rotating antibiotic regimens prevent resistance while managing recurrent symptoms 6

Common Pitfalls to Avoid

• Do not use standard-dose PPIs—systemic sclerosis patients require higher doses than the general population to achieve adequate acid suppression 6
• Do not delay prokinetic therapy in symptomatic patients already on adequate PPI therapy, as combination therapy is more effective than PPI monotherapy 7
• Do not perform fundoplication without considering gastric bypass in patients with severe aperistalsis, as bypass provides better outcomes in this population 3
• Do not neglect the pulmonary implications of inadequately treated reflux, as aspiration directly worsens interstitial lung disease and increases mortality 6