Coexistence of oropharyngeal dysphagia and achalasia in Sjogren's syndrome
Journal
Kaohsiung Journal of Medical Sciences
Journal Volume
38
Journal Issue
6
Pages
607-609
Date Issued
2022
Abstract
We present the case of a 62-year-old woman with Sjogren's syndrome. Within 5 months after the diagnosis of Sjogren's syndrome by a rheumatologist, the patient experienced progressive dysphagia and foreign body sensation after deglutition. She had difficulty eating solid food and easily choked on liquid diet. She complained that the food seemed stuck in the throat after swallowing and that she had to spit out the residue. Gastric acid regurgitation was also observed. The patient presented to our outpatient clinic for evaluation. In the clear water test, multiple swallows, delayed swallow reflex, and post-swallowing choking were noted. Brain magnetic resonance imaging did not reveal stroke or other brain lesions. Chest computed tomography revealed no esophageal mass. In the upper endoscopy, only gastric ulcer and duodenitis, without evident obstructive lesions, were detected. Esophageal biopsy did not indicate eosinophilic infiltration. However, barium esophagography was unsuccessful due to choking and evidence of oral contrast aspiration. A nasogastric tube was placed for feeding because of productive cough and subsequent fever. To further evaluate swallowing, high-resolution impedance manometry (HRIM) was performed using a 36-channel solid-state catheter system with circumferential sensors that were 1 cm apart (Medtronic, Duluth, GA) (Figure 1A,B). The median integrated relaxation pressure (IRP), which is the average lowest pressure through the esophagogastric junction for 4 s within the 10-s window following upper esophageal sphincter relaxation, was 19.2 mmHg (normal <15 mmHg).1 This finding indicated impaired lower esophageal sphincter (LES) relaxation. Accompanied by total esophageal aperistalsis and 20% panesophageal pressurization among the 10 wet swallows in a supine position, type II achalasia was diagnosed based on Chicago classification v3.0.1 Fiberoptic endoscopic evaluation of swallowing (FEES) revealed severe saliva pooling at the bilateral pyriform sinuses before swallowing (Figure 1C) and decreased pharyngeal strength presented as incomplete white-out on swallowing. Immediately after swallowing pudding and dyed thin liquid, penetration/aspiration in the laryngeal vestibule occurred. Furthermore, there were moderate residues at the valleculae and pyriform sinuses based on the Yale Pharyngeal Residue Severity Rating Scale (Figure 1D,E).2 A videofluoroscopic swallowing study demonstrated delayed pharyngeal transit time (normal <1 s) for both thin (1.55 s) and thick (1.08 s) liquid diets. Penetration was also observed during swallowing. These findings indicated that the patient also suffered from coexistence of oropharyngeal dysphagia. Considering the acute onset of oropharyngeal and esophageal dysphagia, intravenous pulse steroid therapy with 500-mg methylprednisolone per day was administered for 3 days, and 5-mg prednisolone qd was maintained. After treatment and swallowing training, dysphagia symptoms improved, and the 6-month HRIM revealed LES relaxation improvement, median IRP reduction to 10.5 mmHg, and esophageal peristalsis and bolus transit recovery (Figure 1F,G). FEES also revealed pharyngeal strength and swallowing function recovery. Swallowing dysfunction in Sjogren's syndrome is categorized into oropharyngeal and esophageal dysphagias. The prevalence of self-reported swallowing dysfunction in Sjogren's syndrome is approximately 60%3; however, these symptoms are frequently attributed to xerostomia alone, without considering oropharyngeal dysphagia and esophageal motility disorders. In Sjogren's syndrome, oropharyngeal dysphagia is attributed to salivary gland dysfunction, mucositis, or neuropathy, whereas esophageal motility disorders are associated with loss of esophageal myenteric plexus attacked by circulating autoantibodies.4 In this case, we used swallowing images and HRIM to confirm the coexistence of these conditions. Thus, comprehensive swallowing assessments and esophageal physiological tests are mandatory in Sjogren's syndrome with swallowing dysfunction to avoid missing oropharyngeal and esophageal dysphagias. Several issues should be considered in this patient. First, the acute course and favorable treatment response is unusual because most patients with achalasia have chronic course and poor response to therapy. Therefore, we conducted a comprehensive diagnostic work-up, including serological, radiological, endoscopic, histological, and functional studies. However, only achalasia was diagnosed by HRIM. The dramatic improvement of dysphagia in this patient could be related to the early intervention with steroid therapy, which might suppress neuronal inflammation and damage caused by unknown antibodies, which is the possible etiology of achalasia. Second, wet swallows in a sitting position during HRIM was not adopted in this case because the normal values for this position were not established in our population; therefore, our motility laboratory still used the Chicago classification v3.0 during that study period. Finally, the new Chicago classification v4.0,5 which adopted the sitting position and provocative maneuvers, may provide more supportive data. Concordant findings in the sitting position and with provocation could significantly increase the confidence to diagnose achalasia in this case. Other differential diagnoses, such as transient esophagogastric junction outflow obstruction, should be considered if any esophageal contraction is observed in the secondary position. Follow-up HRIM with the new classification and long-term observation of this patient's natural course to establish the definite diagnosis is warranted. The authors declare no conflict of interest.
SDGs
Publisher
John Wiley and Sons Inc
Type
letter