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Systemic Sclerosis — SCE Rheumatology MCQ

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ModerateSystemic SclerosisSystemic SclerosisSCE Rheumatology

A 55-year-old woman with limited cutaneous systemic sclerosis has worsening dysphagia for solids and liquids and severe gastro-oesophageal reflux despite a standard-dose proton pump inhibitor. Upper gastrointestinal endoscopy shows reflux oesophagitis but no stricture, obstructing lesion or candidiasis. Oesophageal manometry demonstrates absent peristalsis in the lower two-thirds of the oesophagus and a hypotensive lower oesophageal sphincter. What is the most appropriate next management?

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Correct answer: D — Optimised proton pump inhibitor therapy plus a prokinetic agent

The manometric pattern is typical of systemic-sclerosis oesophageal involvement: distal aperistalsis with a hypotensive lower oesophageal sphincter, causing impaired clearance and severe reflux. Current EULAR recommendations support proton pump inhibitors for SSc-related gastro-oesophageal reflux and consideration of a prokinetic for symptomatic dysmotility. PPI treatment should therefore be optimised, commonly by increasing dose or frequency, with an appropriately selected prokinetic. Oesophageal dilation is indicated for a demonstrated mechanical stricture, which endoscopy has excluded. Fundoplication is not first-line and can aggravate dysphagia in severe aperistalsis; surgery is reserved for carefully selected, medically refractory cases. Mycophenolate does not reliably reverse established oesophageal smooth-muscle dysfunction. Nifedipine may further lower lower-oesophageal-sphincter pressure and worsen reflux.

Reference: European Alliance of Associations for Rheumatology, EULAR recommendations for the treatment of systemic sclerosis: 2023 update, gastrointestinal involvement recommendations, published 2025. https://www.eular.org/document/download/1021/cb11a479-9d7a-4b7e-8020-b8cc1dc69bb8/932