Here is an x-ray of a patient who has just swallowed a solution of barium. A normal "barium swallow" is on the right. Why is there a notch in the esophagus (red arrow)?

How would you characterize the defect on the left? Why is the proximal (upper) esophagus spared? What autoimmune disease is it associated with?

Look at the histology of the muscularis propria of the esophagus and compare it to the normal. How would you characterize the differences?

The 'Special' stains are not H&E, can you tell what the stain is (i.e. what is colored blue)?

Although the vascular changes seen in the histopathology is NOT specifically associated with a collagen vascular disorder, it is characteristic of a disease in a different organ. Do you know what it is?

In addition to dysphagia, the gastroesophageal sphincter tone is compromised. Remembering back to lab 1, what might the result be?

Dysphagia is a common problem among patients with systemic sclerosis. Progressive atrophy and collagenous fibrous replacement of the muscularis are found throughout the alimentary tract, with the lower 2/3 rds of the esophagus most affected. The musculature of the upper 1/3 of the esophagus is skeletal (vs. smooth) which is generally spared from involvement by system sclerosis. In addition to dysphagia, loss of the esophageal sphincter tone can lead to gastrointestinal reflux, esophagitis, Barrett metaplasia, and strictures. The notch seen in both esophagi is due to the aortic arch (as shown in red).

The vascular dilation (varicose) seen in the esophagus are associated with portal hypertension. In addition to system sclerosis, this patient had cirrhosis (which despite its "sclerotic" nature is not typically associated with systemic sclerosis.