Systemic sclerosis

Case contributed by Dr Jan Frank Gerstenmaier

Presentation

Non-specific upper abdominal symptoms

Patient Data

Age: 40 years
Gender: Female

Upper GI barium study

Barium
  1. Single contrast barium swallow in supine/RAO position. There is severe dysmotility of the esophagus and complete failure of emptying into the stomach.
  2. Single contrast barium swallow - now standing up. Due to gravity, barium empties into the stomach.
  3. 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.

LAO: with gas, the gastro-esophageal junction dilates up to 1.5 cm.

Cardiac rosette view.

Frontal: patulous duodenum with delayed gastric/duodenal emptying.

Radiographs of both hands

X-ray

Radiographs of both hands show malalignment of the metacarpophalangeal joints, most marked on the left, in keeping with subluxation. The bone density is normal. There is joint space loss and evidence of erosive arthropathy particularly evident at the metacarpophalangeal joint of the right long finger. Dense soft tissue calcifications are seen in the fingertips 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 1.5 cm. This is borderline normal, and balloon dilatation is usually considered with a cut-off of 1.3 cm. 

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

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