Achalasia Cardia
Conditions
Keywords
Anti-enteric neuronal antibody, Enteric nervous system, Disease Severity, Treatment Outcome, Achalasia Cardia
Brief summary
Achalasia is a rare esophageal motility disorder characterized by impaired relaxation of the lower esophageal sphincter and progressive esophageal dilation. Increasing evidence suggests that autoimmune mechanisms may contribute to its development. Serum anti-enteric neuronal antibodies (AENA) have been reported more frequently in patients with achalasia than in healthy individuals. This study aims to evaluate the association between AENA levels and disease severity in patients with achalasia. Specifically, it aims to determine whether the intensity of AENA positivity is associated with endoscopic severity (measured by the CARS score), esophageal dilation, and integrated relaxation pressure. The study also aims to assess whether AENA status is associated with symptomatic outcomes following peroral endoscopic myotomy (POEM). The objective is to determine whether AENA may serve as a potential biomarker for identifying patients with a more severe disease phenotype and less favorable treatment response.
Interventions
PROCEDUREperoral endoscopic myotomy (POEM)
A subset of eligible patients subsequently underwent POEM at our institution using the standardized technique described by Inoue et al. Postoperative follow-up assessments were conducted at 1, 3, and 6 months and included evaluation of Eckardt scores. At the 6-month follow-up, repeat esophagogastroduodenoscopy (with grading of erosive esophagitis according to the Los Angeles classification esophagitis), HRM, and barium esophagogram were performed.
Sponsors
Peking Union Medical College Hospital
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to 75 Years
Inclusion criteria
* Age between 18 and 75 years; * Fulfilment of the diagnostic criteria for AC according to the Chicago Classification version 4.0 * Completion of symptomatic assessment, esophagogastroduodenoscopy, high-resolution manometry (HRM), and barium esophagram.
Exclusion criteria
* pregnant * lactating * with coexisting major gastrointestinal disorders
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Proportion of Participants with Positive Serum Anti-Enteric Neuronal Antibodies (AENA) | Baseline | Serum AENA is assessed by indirect immunofluorescence using guinea pig intestinal submucosal whole-mount preparations. Patient sera are incubated with neuronal tissue, followed by fluorescein-conjugated secondary antibodies. Mouse anti-Hu antibody serves as a positive control and PBS as a negative control. Fluorescence intensity is graded by independent investigators as: 0 (negative), 1 (moderate positive), or 2 (strong positive). This measure reports the percentage of participants graded as 1 or 2 (AENA-positive rate). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| The Eckardt score | Baseline, 1-month follow-up after the procedure, 6-month follow-up after the procedure | The Eckardt score was used to evaluate symptom severity in patients with AC. This composite score includes four components: dysphagia, retrosternal pain, regurgitation, and weight loss. Dysphagia, retrosternal pain, and regurgitation are each graded from 0 to 3 (0 = none; 1 = occasional; 2 = daily; 3 = at each meal). Weight loss is also scored from 0 to 3 (0 = none; 1 = \< 5 kg; 2 = 5-10 kg; 3 = \> 10 kg). The total score ranges from 0 to 12, with higher scores indicating greater symptom severity. |
| Endoscopic evaluation | Baseline, 6-month follow-up | Endoscopic assessment was conducted using the CARS scoring system, a validated tool developed through video-based reliability testing and shown to correlate with disease severity. The CARS system allocates 0-2 points each for the domains of Contents, Anatomy, and Lower Esophageal Sphincter (LES) Resistance, and 1 point for each Stasis component. Erosive esophagitis was graded according to the Los Angeles classification into four severity grades. |
| High-resolution manometry (HRM) | Baseline, 6-month follow-up | HRM was conducted per standard protocol, and key parameters were analyzed following the Chicago Classification version 4.0 criteria, including AC subtype, integrated relaxation pressure (IRP), basal LES pressure (BLESP), and distal contractile integral (DCI); and Radiologic assessment: A barium esophagogram was used to measure the maximum barium column width and assess esophageal tortuosity. |
| The degree of esophageal distortion on barium esophagogram | Baseline, 6-month follow-up | The degree of esophageal distortion on barium esophagogram was classified into four grades: None, mild, moderate, and severe. Mild distortion was defined as slight dilation or localized tortuosity with mild distal narrowing. Moderate distortion was characterized by a "C"-shaped or partial "S"-shaped configuration, whereas severe distortion included "L"-type or sigmoid-type esophagus. Senior gastroenterologists and radiologists with over 10 years of experience determined the grading. |
Countries
China
Outcome results
None listed