Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices

Abstract

A previously unseen manifestation of primary biliary cholangitis with portal hypertension and a new type of esophageal ulcer that should be explored as a possible alternate source of bleeding in PBC patients to expedite early recognition and treatment. We present a case of a 71-year-old Caucasian female with PBC presented with hematemesis. EGD revealed cratered and linear ulcers with irregular shapes and blood intermittently oozing from the center in the esophagus in between >5mm non-bleeding esophageal varices in the middle and lower one third of the esophagus. Bipolar cautery was used successfully to halt bleeding from the ulcers and she was discharged with appropriate outpatient follow up with recommendations of non-selective beta blockers and follow-up EGD. Our patient had unexpected and unusual cause of hematemesis in the form of multiple esophageal ulcers in-between variceal columns. Nonvariceal bleeding in the presence of varices is extremely uncommon in PBC. Cirrhosis causing portal hypertension can cause an abnormally enlarged and thickened submucosal veins known as varices. In events of ischemia, these thickened veins cannot compensate further as they have reached maximal dilatation and have minimal reserve, thus resulting in ulcers. Other differentials such as endoscopic band ligation, sclerotherapy, recent nasogastric tube placement, stress ulcers, caustic ingestion, antibiotics use, chronic non-steroidal anti-inflammatory drugs (NSAID) usage, CMV, HSV, Candida, GERD, or Behcet's were ruled out. Given that upon endoscopic examination, the esophageal ulcers were found to bleeding instead of the varices, they should be considered in the differential in the etiology of hematemesis in primary biliary cirrhosis and thoroughly searched for during an endoscopic procedure for early treatment and subsequent secondary prevention.1813_A Figure 1. Esophageal Ulcers and Varices in Primary Biliary Cholangitis1813_B Figure 2. Ulcers in between Esophageal Varices