High-resolution manometry in patients with idiopathic inflammatory myopathy: Elevated prevalence of esophageal involvement and differences according to autoantibody status and clinical subset.

INTRODUCTION: In this study we assessed high-resolution manometry (HRM) findings in patients with dermatomyositis and polymyositis. METHODS: From 2008 to 2015, we performed a cross-sectional study of myositis patients. A survey of esophageal symptoms and HRM data were analyzed and compared among different clinical and serologic groups. RESULTS: Twenty-four (45%) of the 53 patients included in the study had manometric involvement that was not correlated with any esophageal symptom (P = 0.8). Failed waves (34% vs. 0%, P = 0.004) and decreased upper esophageal sphincter pressure (50 vs. 70 mm Hg, P = 0.03) were more common in polymyositis than in dermatomyositis patients. Jackhammer esophagus was more common in anti-TIF1-γ patients (30% vs. 9%, P = 0.04), and lower esophageal sphincter involvement (47% vs. 25%, P = 0.03) was more prevalent in patients with the antisynthetase syndrome. CONCLUSIONS: Esophageal involvement is common in myositis patients, but it correlates poorly with esophageal symptoms. Specific clinical and serologic groups have different manometric features. Muscle Nerve 56: 386-392, 2017.

Endoscopic findings of laryngopharyngeal and esophageal involvement in autoimmune bullous disease.

BACKGROUND AND AIM: Autoimmune bullous disease (ABD) is induced by autoantibodies against cell adhesion molecules, and blistering may occur on the mucous membranes of the eyes, nose, mouth, oral cavity, laryngopharynx, and esophagus. Endoscopic prevalence and features of ABD-associated esophageal lesions are not well known. We conducted the present study to assess the endoscopic prevalence of ABD-associated mucosal lesions. METHODS: Endoscopic prevalence of mucosal lesions, particularly laryngopharyngeal and esophageal lesions, was used as the primary endpoint to assess the significance of upper gastrointestinal endoscopy, and clinical and endoscopic features were secondary endpoints. RESULTS: Of 123 ABD patients, 50.4% had apparent oral or laryngopharyngeal lesions and 30.8% had laryngopharyngeal lesions. Esophageal lesions were detected through normal observation in 16.8% of affected patients, whereas 40.6% exhibited epidermolysis or blood blisters by mechanical inducement, regardless of esophageal mucosal lesion detection by normal observation. Additionally, 56.0% exhibited the Nikolsky sign with mechanical inducement. Of the 123 patients, 29.2% did not have exposed skin lesions. Of these patients, 77.7% had oral cavity or laryngopharyngeal lesions, 36.1% had esophageal lesions, and 58.3% exhibited the Nikolsky sign on esophageal mucosa. CONCLUSION: It is important to determine the endoscopic characteristics and findings of ABD. ABD can be suspected from endoscopic findings.

Esophageal Involvement in Multiple System Atrophy.

The prevalence of esophageal involvement and its impact on clinical manifestations in patients with multiple system atrophy (MSA) remains unknown. We recruited 16 consecutive patients with dysphagia associated with MSA (MSA group) and 16 consecutive patients with dysphagia associated with amyotrophic lateral sclerosis (ALS group). We assessed the presence or absence of food stagnation within the esophagus using videofluorography. Food stagnation within the esophagus was observed in 16 patients (100 %; 7 severe, 9 mild) in the MSA group and in 4 patients (25 %; 4 mild) in the ALS group (P < 0.001). Follow-up videofluorography revealed that food stagnation in patients with MSA could exacerbate during the disease course. Patients with MSA and severe food stagnation showed a wide range of intraesophageal stasis by videofluorography. Among the 16 patients in the MSA group, 4 developed aspiration pneumonia and 1 died of suffocation associated with food regurgitation during continuous positive airway pressure therapy. In conclusion, food stagnation within the esophagus occurs more frequently in MSA patients with dysphagia than in ALS patients with dysphagia. Because food stagnation can cause serious complications such as aspiration pneumonia and suffocation, patients with MSA should be evaluated by videofluorography, especially those with stagnation in the esophageal phase.

Journey into the Esophageal Complications: Decoding Systemic Sclerosis with Cutting-Edge Endoscopy, Manometry, and Ambulatory pH-Study.

Purpose: Systemic Sclerosis (SSc) is a rare connective tissue disorder characterized by autoimmunity, fibrosis, and vasculopathy that affects the skin and internal organs, including the gastrointestinal tract, particularly the esophagus. This article highlights the characteristics and clinical symptoms of esophageal involvement in patients with SSc. Patients and Methods: This study was conducted between November 2022 to August 2023, including 26 already diagnosed cases of SSc in the Department of Rheumatology and Rehabilitation and Kurdistan Center for Gastroenterology and Hepatology-Sulaymaniyah, Iraq. Esophageal involvement was investigated using esophageal manometry, esophagogastroduodenoscopy (EGD), and 24-hour impedance-pH monitoring. Results: Females were significantly predominant (P = 0.019) regarding the symptoms; 76.9% of the patients had heart burn, 76.9% dysphagia, 73.1% water brush, and 69.2% regurgitation. In total, 69.2% of the patients showed erosive gastrointestinal reflux disease (GERD) on EGD, 76.9% had decreased lower esophageal sphincter pressure (DLESP) and decreased distal esophageal peristaltic contractions (DDEPC) on esophageal manometry, and 84.6% had reflux on pH monitoring. Raynaud's phenomenon is the most common and typically the earliest clinical manifestation of SSc. The presence of erosive GERD was found to significantly increase the risk of developing dysphagia (B = 4.725, P = 0.014, OR = 3.482) and regurgitation (B = 3.521, P = 0.006, OR = 4.030). Conclusion: It is crucial to take gender-specific considerations into account when diagnosing and managing esophageal complications in patients with systemic sclerosis (SSc). Additionally, employing various diagnostic assessments to detect esophageal involvement during SSc is essential. Erosive GERD has been identified as a risk factor that contributes to the development of dysphagia and regurgitation in individuals with SSc.

Accuracy of the endoscopic evaluation of esophageal involvement in esophagogastric junction cancer.

BACKGROUND: Esophageal involvement length (EIL) is a promising indicator of metastasis or recurrence in mediastinal lymph nodes (MLNs) in adenocarcinoma of the esophagogastric junction (EGJ). This study aimed to elucidate the accuracy of the preoperative endoscopic evaluations of EIL and its clinical significance in this disease. MATERIALS AND METHODS: In total, 75 patients with Siewert type II (N = 53) or III (N = 22) adenocarcinoma of the EGJ, who underwent surgical resection without preoperative therapy between 1995 and 2016 were enrolled. We retrospectively examined the accuracy of the preoperative endoscopic evaluations of EIL (preoperative EIL), compared to the pathologically evaluated EIL. Finally, we investigated the association between preoperative EIL and metastasis or recurrence in MLNs. RESULTS: The accuracy of the preoperative EIL within a 1-cm interval was only 53.3%. Among patients with discordance between the pre- and postoperative evaluations, 68.6 % had the underestimation in the preoperative EIL. pN1-3 (OR = 5.85, 95% CI: 1.03-33.17) and undifferentiated histologic type (OR = 2.52, 95% CI: 0.89-7.14) were potential risk factors for the discordance. Regarding metastasis or recurrence in MLNs, preoperative EIL of 2-3 cm (OR = 10.41, 95% CI: 1.35-80.11) and >3 cm (OR = 8.33, 95% CI: 1.09-63.96) were independent predictors. CONCLUSION: Although the accuracy of the endoscopic evaluations of EIL is insufficient with many underestimations, EIL should be assessed in preoperative staging because of significant predictive power for metastasis or recurrence in MLNs.

Esophageal high-resolution impedance manometry alterations in asymptomatic patients with systemic sclerosis: prevalence, associations with disease features, and prognostic value.

This study aims to investigate pre-clinical esophageal involvement in systemic sclerosis (SSc) by high-resolution impedance manometry (HRiM), its associations with disease features including lung involvement, and its predictivity of esophageal symptoms overtime. Charts of 45 asymptomatic (no heartburn/regurgitation/dysphagia) SSc patients (96% females; mean age 46 years) with at least one follow-up (FU) visit and complete clinical, serological, functional, and radiological assessment, including high-resolution computed tomography (HRCT) of the chest and lung function tests, that had undergone esophageal HRiM were retrospectively evaluated. Esophagogastric junction-contractile integral (EGJ-CI) and esophageal body motility, as evaluated by mean distal contractile integral (DCI), were assessed. SSc patients had a normal esophageal motility in 7/45 cases, a defective EGJ-CI in 28, an ineffective esophageal motility (IEM) in 17, and aperistalsis in 12. Defective EGJ-CI was associated with IEM/aperistalsis in 20 cases, while 9 patients had isolated IEM. Defective EGJ-CI and/or IEM/aperistalsis were associated with a diffusing lung capacity for CO < 80% of predicted value (all p < 0.05), while defective EGJ-CI was also associated with interstitial lung disease on HRCT (p = 0.03). Prevalence of any HRiM abnormality was higher in anti-centromere antibody negative patients (all p < 0.05). IEM/aperistalsis independently increased the risk of esophageal symptoms by 2.3-fold (95% CI 1.1-5.7) and was associated with their higher cumulative incidence with respect to patients with other HRiM patterns at FU (χ2 = 4.63; p = 0.03). SSc patients asymptomatic for esophageal involvement can have HRiM abnormalities in up to 84% of cases. A baseline-impaired motility is a risk factor for symptomatic esophageal disease.

Esophageal involvement of pemphigus vulgaris associated with upper gastrointestinal bleeding.

Esophageal involvement of pemphigus vulgaris is rare, and when present, the most common presenting symptoms reported in the medical literature are odynophagia and dysphagia. Here, we present two cases of pemphigus vulgaris presenting with upper gastrointestinal hemorrhage because of esophageal involvement of the disease. In case 1, a 41-year-old female patient with a prior diagnosis of pemphigus vulgaris presented with hematemesis. Esophagogastroduodenoscopy showed diffuse mucosal exfoliation and oozing bleeding of the oropharynx and esophagus. The patient recovered after the administration of high-dose corticosteroids and immunosuppressants. In case 2, a 30-year-old female patient with known pemphigus vulgaris also presented with hematemesis, showing similar endoscopic findings to the first case. She also responded to the same treatment. Esophageal involvement of pemphigus vulgaris responds to high-dose corticosteroids and immunosuppressants. Thus, in patients with pemphigus vulgaris with signs or symptoms of upper gastrointestinal bleeding, an early endoscopy for the evaluation of esophageal involvement is beneficial.

Esophageal Involvement of Pemphigus Vulgaris Associated with Upper Gastrointestinal Bleeding

Esophageal involvement of pemphigus vulgaris is rare, and when present, the most common presenting symptoms reported in the medical literature are odynophagia and dysphagia. Here, we present two cases of pemphigus vulgaris presenting with upper gastrointestinal hemorrhage because of esophageal involvement of the disease. In case 1, a 41-year-old female patient with a prior diagnosis of pemphigus vulgaris presented with hematemesis. Esophagogastroduodenoscopy showed diffuse mucosal exfoliation and oozing bleeding of the oropharynx and esophagus. The patient recovered after the administration of high-dose corticosteroids and immunosuppressants. In case 2, a 30-year-old female patient with known pemphigus vulgaris also presented with hematemesis, showing similar endoscopic findings to the first case. She also responded to the same treatment. Esophageal involvement of pemphigus vulgaris responds to high-dose corticosteroids and immunosuppressants. Thus, in patients with pemphigus vulgaris with signs or symptoms of upper gastrointestinal bleeding, an early endoscopy for the evaluation of esophageal involvement is beneficial.