Diagnosis

Systemic sclerosis

Findings

Axial computed tomography (CT) image demonstrates a patulous distal esophagus and fibrosis at the lung bases.

Discussion

Systemic sclerosis is a collagen vascular disease with a 3:1 female to male predominance. The gastrointestinal (GI) tract is affected in up to 90% of cases. The esophagus is the most common organ of GI tract involvement. The upper one third of the esophagus is typically spared as it contains skeletal muscle, while the distal two thirds of the esophagus is composed of smooth muscle. Systemic sclerosis results in collagen deposition in the lamina propria and submucosa. As a result, the lower esophageal sphincter can become incompetent with resultant gastroesophageal reflux and distal esophageal strictures. Patients with systemic sclerosis are at increased risk for Barrett’s esophagus.

Pulmonary involvement is reported in up to 80% of cases. Interstitial lung disease initially manifests as reticular opacities that can progress to honeycombing in the later stages. The fibrosis has a peripheral and basilar predominance, as seen in the above case. Patients with systemic sclerosis have a higher risk of lung cancer than the general population.

1. Kim EA, Lee KS, Johkoh T, et al. Interstitial lung diseases associated with collagen vascular diseases: Radiologic and histopathologic findings. Radiographics. 2002;22:S151-S165.

2. Pickhardt PJ. The “hide-bound” bowel sign. Radiology. 1999;213:837-838.