Pauci-Eosinophilic Esophagitis: An Under-Recognized Variant of Eosinophilic Esophagitis.
Shriram Jakate. Rush University Medical Center, Chicago, IL
Background: Symptomatic patients with eosinophilic esophagitis (EE) characteristically present with dysphagia or food impaction. Endoscopically, these patients may show ringed, furrowed or strictured esophagus and tiny white spots involving any part of esophagus but preferentially the mid esophagus. Histologically, markedly increased mucosal eosinophils, >20/hpf, are considered essential for making the diagnosis and differentiating EE from GERD. We describe a variant where the eosinophils are considerably fewer (<10/hpf) than expected, but the endoscopic and other histological features and clinical aspects are similar to classical EE. Failure in recognition of this variant may lead to therapeutic mismanagement.
Design: The endoscopy and clinical database records in our institution were reviewed to select all clinically and endoscopically suspected EE cases from 2002 to 2010 that had biopsy sampling of mid and distal esophagus (total of 208 biopsy events). These included 171 initial biopsies (125 M, 46 F, mean age 23) and post-therapeutic follow-up biopsies in 37/171 patients. Their histological features, endoscopic findings and clinical features were reviewed.
Results: All patients had dysphagia and/or food impaction and many of the following endoscopic findings such as rings, furrows, strictures, minute white spots and preferential mid esophageal involvement. 162/171 (95%) patients with initial biopsies showed histological features of classical EE such as severe basal zone hyperplasia, markedly increased eosinophils (>20/hpf) and focal luminal eosinophilic clusters. 2/171 (1%) patients showed histologically normal squamous mucosa (the endoscopic changes were reinterpreted as spasmodic). 8/171 (4%) patients showed marked basal zone hyperplasia and patchy areas of abrupt keratinization but eosinophils were sparse and maximum of 10/hpf in focal areas. These cases were diagnosed as PEE. Of 37 patients who underwent post-therapeutic endoscopic examination and biopsies, 24/31 (77%) of classical EE and 5/6 (83%) PEE patients showed marked and almost complete histological and endoscopic resolution.
Conclusions: PEE is a variant that has similar clinical presentation and endoscopic findings as EE but lacks the level of eosinophilia that is considered essential for a histological diagnosis of EE. This variant needs recognition since it is unlikely to be histologically diagnosed as EE, but can be clinically managed similar to classical EE. Furthermore, it is important that dilation of a stricture is either avoided or performed cautiously in PEE similar to EE, given the potential risk of fracture.
Monday, February 28, 2011 1:00 PM
Poster Session II # 79, Monday Afternoon