1765 Delayed Esophageal Clearance Plays an Important Role in Reflux Symptomology in Scleroderma Patient

Abstract

INTRODUCTION: We present the case of a patient with advanced scleroderma who experienced symptoms of refractory reflux despite maximum acid suppressing therapy. The use of 24-hour ambulatory esophageal pH impedance measurement led to the diagnosis of esophageal stasis, with subsequent management leading to the improvement of symptoms even with decreased proton pump inhibitor (PPI) dose. CASE DESCRIPTION/METHODS: 70-year old female with history of advanced scleroderma presented with severe retrosternal burning which she described as worst at night. She was taking 60mg Dexlansoprazole daily, an aluminum-magnesium-antacid, as well as an H2 blocker. An outside CT of the chest and abdomen showed a fluid-filled dilated esophagus and upper endoscopy was significant for a small hiatal hernia. The patient initially underwent high resolution manometry (HRM) which showed absent peristalsis. Subsequent pH impedance study identified low impedance in the esophagus with good gastric acid control (gastric acid exposure of 36%) but markedly abnormal gastric acid exposure in the distal esophagus: recumbent time acid exposure 94%, upright acid exposure 3%, and total acid exposure time of 40% despite high dose PPI. Recumbent position acid exposure at the distal esophagus was more prolonged than nocturnal gastric acidity, indicating esophageal stasis was a major factor in symptomatology. The patient was instructed to take her PPI and antacids close to bedtime, sleep with head elevation, and avoid late meals. The patient experienced significant symptom improvement and her PPI dose was successfully decreased. DISCUSSION: Scleroderma is a multiorgan connective tissue disorder affecting the gastrointestinal tract. The most commonly affected portion of the GI tract is the esophagus, with destruction of the smooth muscles of the lower esophageal sphincter and distal esophageal body. Abnormal reflux has been identified in patients with scleroderma on 24-hour pH monitoring in as many as 80% of patients. However, these study findings do not account for delayed esophageal clearance from failed or ineffective esophageal contractions. Patients with systemic sclerosis frequently have large dilated esophagi (pseudoachalasia) with subsequent stasis a major contributing factor for their symptoms. Thus, while pH studies are a useful diagnostic tool in patients with advanced scleroderma, therapeutic measures addressing esophageal stasis, such as nighttime head elevation, should be encouraged.