Clinical and Manometric Characteristics of Patients with Achalasia: One Disease with Three Presentations or Three Diseases with One Presentation?

Background And Aims: The three manometric patterns of achalasia are considered by some authors as different stages in the evolution of the same disorder. The aims of our study were to characterize patients with achalasia, in order to find key differences supporting the idea of progression from one type to the other, and to assess the clinical evolution in time.

Methods: From 280 high resolution esophageal manometry recordings we selected unique patients with achalasia. A standardized questionnaire used prior to each manometry recorded their symptoms. Manometric parameters (resting lower esophageal sphincter (LES) pressure, 4s-integrated relaxation pressure (IRP), length of the esophagus, etc.) were recorded. Patients were contacted to establish the clinical evolution.

Results: We identified 108 new achalasia cases (mean age 48.2±16.2 years, 52.8% type I, 42.6% type II), 52 (48.1%) women. Dysphagia (98.1%), cough (64.8%), belching (60.2%) and reflux symptoms (53.7%) were frequently reported. Patients with type I achalasia reported more often that dysphagia worsened, compared to type II patients (χ2=7.3, p =0.007). Age, duration of dysphagia, body mass index (p=0.067) and esophageal length were similar in type I and type II achalasia. Resting LES pressure (64.7±22.6 mmHg vs. 54.3±21.6 mmHg, p=0.019) and 4s-IRP (45.3±17.6 mmHg vs. 38.4±15.5 mmHg, p=0.036) were higher in type II compared to type I achalasia. Overweight patients had a lower LES resting pressure and 4s-IRP compared to lean subjects. After a mean follow-up of 36.8±13.4 months, 49 (45.3%) patients responded to our follow-up, and 77.5% had an Eckardt score ≤ 3.

Conclusions: Type I achalasia was the most common in our group. Type I patients had lower BMI but similar duration of dysphagia and mean age compared to type II. Type III is seldom and present in older patients. These findings suggest low probability of progression from type III and II to type I achalasia. Patients with type II achalasia had higher resting LES pressure and 4s-IRP than type I achalasia patients.