Achalasia: incidence, prevalence and survival. A population-based study.

BACKGROUND Studies of achalasia epidemiology are important as they often yield new insights into disease etiology. In this study, our objective was to carry out the first North American population-based study of achalasia epidemiology using a governmental administrative database. METHODS All residents in the province of Alberta, Canada receive universal healthcare coverage as a benefit. The provincial health ministry, Alberta Health and Wellness, maintains a central stakeholder database of patient demographic information and physician billing claims. We defined an achalasia case as a billing claim submitted for the years 1996-2007 with an ICD-9-CM code of 530.0 or 530 and a Canadian Classification of Procedure treatment code of 54.92A (endoscopic balloon dilation) or 54.6 (esophagomyotomy). A preliminary validation study of the case definition demonstrated a sensitivity of 85% and specificity of 99% for known cases and controls. KEY RESULTS A total of 463 achalasia cases were identified from 1995 to 2008 (59.6% males). Mean age at diagnosis was 53.1 years. In 2007, the achalasia incidence was 1.63/100,000 (95% CI 1.20, 2.06) and the prevalence was 10.82/100,000 (95% CI 9.70, 11.93). We observed a steady increase in the overall prevalence rate from 2.51/100,000 in 1996 to 10.82/100,000 in 2007. Survival of achalasia cases was significantly less than age-sex matched population controls (P < 0.0001). CONCLUSIONS & INFERENCES Using a population-based approach, the incidence and prevalence of treated achalasia is 1.63/100,000 and 10.82/100,000, respectively. The disease appears to have a stable incidence but a rising prevalence. Survival of achalasia cases is significantly less than age-matched healthy controls.