Esophageal Manifestations of Dermatological Diseases, Diagnosis and Management.

Purpose of Review: The purpose of this article is to discuss the diagnosis and treatment of diseases that affect both the skin and the esophagus. Recent Findings: The diagnosis of dermatological conditions that affect the esophagus often requires endoscopy and biopsy with some conditions requiring further investigation with serology, immunofluorescence, manometry, or genetic testing. Many conditions that affect the skin and esophagus can be treated successfully with systemic steroids and immunosuppressants including pemphigus, pemphigoid, HIV, esophageal lichen planus, and Crohn's disease. Many conditions are associated with esophageal strictures which are treated with endoscopic dilation. Furthermore, many of the diseases are pre-malignant and require vigilance and surveillance endoscopy. Summary: Diseases that affect the skin and esophagus can be grouped by their underlying etiology: autoimmune (scleroderma, dermatomyositis, pemphigus, pemphigoid), infectious (herpes simplex virus, cytomegalovirus, human immunodeficiency virus), inflammatory (lichen planus and Crohn's disease), and genetic (epidermolysis bullosa, Cowden syndrome, focal dermal hypoplasia, and tylosis). It is important to consider primary skin conditions that affect the esophagus when patients present with dysphagia of unknown etiology and characteristic skin findings.

Chicago Classification of esophageal motility disorders: Past, present, and future.

The Chicago Classification (CC) is a dynamic, evolving classification scheme created by a diverse group of international esophageal experts. Its application has transformed the way esophageal motor data are used to define motility disorders, each iteration seeking to advance, simplify, and standardize the way clinicians worldwide diagnose esophageal dysmotility. The most recent update, CC version 4.0 (CCv4.0), emphasizes the importance of clinical context and distinguishes clinically relevant, conclusive manometric diagnoses from irrelevant manometric observations. Future iterations of CC may refine the classification of spastic esophageal disorders and incorporate machine learning and physics-based modeling to improve metrics.

Diagnostic yield and reliability of post-prandial high-resolution manometry and impedance-ph for detecting rumination and supragastric belching in PPI non-responders.

BACKGROUND: Supragastric belching (SGB) and rumination are behavioral disorders associated with proton pump inhibitor (PPI) non-response and can be diagnosed using multichannel intraluminal impedance-pH (MII-pH) and post-prandial high-resolution impedance manometry (PPHRIM). This pilot study compared diagnostic yield and inter-rater agreement for SGB and rumination using MII-pH and PPHRIM. METHODS: Three esophageal physiologists performed blinded interpretations of MII-pH and PPHRIM in 22 PPI non-responders. Raters selected from 4 diagnostic impressions (normal, GERD, behavioral disorders, GERD+behavioral disorders) without clinical context. Primary outcomes were diagnostic impressions compared against clinical gold standard impression, between raters, and between test modalities. Following a 28-month wash-out period, raters re-interpreted MII-pH with clinical context and under consensus definition of diagnostic criteria. KEY RESULTS: Compared to gold standard, rater accuracy for presence of behavioral disorders ranged from 45 to 77% on MII-pH and 45-59% on PPHRIM. On MII-pH, inter-rater agreement was fair for diagnosis (ĸ0.32, p < 0.01) and suboptimal for presence of behavioral disorders (ĸ0.13, p = 0.14). On PPHRIM, inter-rater agreement was suboptimal for both diagnosis (ĸ0.03, p = 0.34) and presence of a behavioral disorder (ĸ-0.22, p = 0.96). Inter-rater agreement improved in post hoc MII-pH interpretations. Rumination was more frequently identified on PPHRIM (23, 35%) compared to MII-pH (7, 11%). CONCLUSIONS AND INFERENCES: Diagnostic accuracy and inter-rater agreement are higher for MII-pH than PPHRIM, and behavioral disorders are more frequently identified on PPHRIM. Identifying behavioral disorders on MII-pH and PPHRIM has implications for clinical evaluation of PPI non-response; clinical context is essential for accurate study interpretation. Further work is needed to standardize definitions and interpretations.

Esophageal Manometry Competency Program Improves Gastroenterology Fellow Performance in Motility Interpretation.

INTRODUCTION: Competency-based medical education (CBME) for interpretation of esophageal manometry is lacking; therefore, motility experts and instructional designers developed the esophageal manometry competency (EMC) program: a personalized, adaptive learning program for interpretation of esophageal manometry. The aim of this study was to implement EMC among Gastroenterology (GI) trainees and assess the impact of EMC on competency in manometry interpretation. METHODS: GI fellows across 14 fellowship programs were invited to complete EMC from February 2018 to October 2018. EMC includes an introductory video, baseline assessment of manometry interpretation, individualized learning pathways, and final assessment of manometry interpretation. The primary outcome was competency for interpretation in 7 individual skill sets. RESULTS: Forty-four GI trainees completed EMC. Participants completed 30 cases, each including 7 skill sets. At baseline, 4 (9%) participants achieved competency for all 7 skills compared with 24 (55%) at final assessment (P < 0.001). Competency in individual skills increased from a median of 4 skills at baseline to 7 at final assessment (P < 0.001). The greatest increase in skill competency was for diagnosis (Baseline: 11% vs Final: 68%; P < 0.001). Accuracy improved for distinguishing between 5 diagnostic groups and was highest for the Outflow obstructive motility disorder (Baseline: 49% vs Final: 76%; P < 0.001) and Normal motor function (50% vs 80%; P < 0.001). DISCUSSION: This prospective multicenter implementation study highlights that an adaptive web-based training platform is an effective tool to promote CBME. EMC completion was associated with significant improvement in identifying clinically relevant diagnoses, providing a model for integrating CBME into subspecialized areas of training.

Long-term Continued Proton Pump Inhibitor Use is Common in Patients Diagnosed with Eosinophilic Esophagitis Despite Failure of Histologic Response: Data from a two-centre study: Long-term PPI Use in Patients with EoE.

Background and Aims: Treatment paradigms for proton pump inhibitors (PPIs) in eosinophilic esophagitis (EoE) are evolving. We aimed to determine patterns of long-term PPI use after EoE diagnosis in PPI histologic non-responders. Methods: We conducted a retrospective review at University of Colorado (UCH) and University of North Carolina (UNC) of EoE patients who were histologic non-responders to PPIs. Data were extracted from electronic medical records related to demographics, PPI use, and reasons for continuing or stopping PPI. Results: Of 67 patients in the UCH cohort, PPIs were initially discontinued in 9 (13%). Of 58 remaining on PPI, 48% were not instructed to discontinue therapy and 26% continued for symptom improvement. Of 675 patients at UNC, PPI was stopped in 185 (27%). Of patients remaining on PPI, 15% were not told to discontinue therapy and 62% were continued for symptom improvement. At last contact, >50% of patients remained on PPI at both centres with most common reasons for continuation being symptom improvement and not telling patients to discontinue. In the UNC cohort, clinical features associated with remaining on PPI included children younger than 18 years (p=0.01), males (p<0.001), heartburn symptoms (p<0.001) and hiatal hernia (p=0.004). Patients with dysphagia were less likely to remain on PPIs (p<0.001). Conclusions: Long-term PPI use is common in EoE patients even without histologic response. Failure to instruct patients to discontinue therapy was a common reason for long-term use, thus PPI use should be revisited in all EoE patients to confirm clinical benefit.

Proton Pump Inhibitor-Refractory Gastroesophageal Reflux Disease.

Proton pump inhibitor (PPI)-refractory gastroesophageal reflux disease (GERD) is defined by the presence of troublesome GERD symptoms despite PPI optimization for at least 8 weeks in the setting of ongoing documented pathologic gastroesophageal reflux. It arises from a dysfunction in protective systems to prevent reflux and as propagation of physiologic reflux events. Treatment possibilities include pharmacologic options, invasive management strategies, and endoluminal therapies. Management strategy should be personalized to the patient's needs and mechanistic dysfunction. This article reviews the definition, mechanisms, and management options for PPI-refractory GERD.