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Scleroderma Oesophagus — RACP Adult Medicine MCQ

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ModerateRheumatologyScleroderma OesophagusRACP Adult Medicine

A 48-year-old woman with known systemic sclerosis develops progressive dysphagia to solids and liquids. Oesophageal manometry shows aperistalsis of the distal two-thirds of the oesophagus with a hypotensive lower oesophageal sphincter. What is the most appropriate management?

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Correct answer: B — Heller myotomy

Scleroderma oesophagus is characterised by smooth muscle dysfunction (distal 2/3 aperistalsis + hypotensive LES), causing dysphagia and severe GORD. Management focuses on aggressive acid suppression with high-dose PPI (to prevent peptic stricture and Barrett's) plus lifestyle measures (upright posture after meals, small frequent meals). Prokinetics (metoclopramide, domperidone) may help with gastric emptying. Pneumatic dilation and myotomy are for achalasia, not scleroderma oesophagus. There is no treatment to restore peristalsis.

Reference: eTG – 2025 – Gastrointestinal; EULAR – 2023 – SSc GI Management