Systemic sclerosis

Case contributed by Dr Jan Frank Gerstenmaier

Presentation

This female patient has been complaining of non-specific upper abdominal symptoms.

Patient Data

Age: 40
Gender: Female
Barium

Upper GI barium study

Double contrast barium swallow. The upper and mid-sections of the esophagus are patulous and again show severe dysmotility. There is smooth tapering towards the gastro-esophageal junction. No convincing stricture. No mucosal abnormality.

X-ray

Radiographs of both hands

Radiographs of both hands show abnomal alignment of the metacarpophalangeal joints, most marked on the left, in keeping with subluxation. The bone density appears normal. There is joint space loss and evidence of erosive arthropathy particularly evident at the metacarpophalangeal joinft of the right long finger. Dense soft tissue calcifications are seen in the finger tips and along the ulnar aspect of the right wrist/distal forearm.

Case Discussion

The main abnormality here is severe esophageal dysmotility. Other typical features shown here are the patulous esophagus, smooth tapering, patulous duodenum and delayed duodenal/gastric emptying. Severe reflux was also demonstrated (no images). The gastro-esophageal junction measured 1.5cm. This is borderline normal, and ballon dilatation is usually considered with a cut-off of 1.3cm. 

Companion radiographs of both hands in this patient show typical dystrophic soft tissue calcifications and arthropathy as described.

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Case information

rID: 23125
Published: 23rd May 2013
Last edited: 12th Feb 2019
Inclusion in quiz mode: Included

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