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The advent of the Chicago Classification for esophageal motility disorders allowed for clinically reproducible subgrouping of patients with achalasia based on manometric phenotype. However, there are limited data with regards to racial variation using high-resolution esophageal manometry (HREM). The aim of our study was to evaluate the racial differences in patients with achalasia diagnosed with HREM using the Chicago Classification. We evaluated the clinical presentation, treatment decisions and outcomes between blacks and non-blacks with achalasia to identify potential racial disparities.We performed a retrospective review of consecutive patients referred for HREM at a single tertiary referral center from June 2008 through October 2012. All patients diagnosed with achalasia on HREM according to the Chicago Classification were included. Demographic, clinical and manometric data were abstracted. All studies interpreted before the Chicago Classification was in widespread use were reanalyzed. Race was defined as black or non-black. Patients who had missing data were excluded. Proportions were compared using chi-squared analysis and means were compared using the Student’s t-test.A total of 1,268 patients underwent HREM during the study period, and 105 (8.3%) were manometrically diagnosed with achalasia (53% female, mean age: 53.8 ± 17.0 years) and also met the aforementioned inclusion and exclusion criteria. A higher percentage of women presented with achalasia in blacks as compared to whites or other races (P < 0.001). Non-blacks were more likely to present with reflux than blacks (P = 0.01), while blacks were more likely to be treated on the inpatient service than non-blacks (P < 0.001). There were no other significant differences noted in clinical presentation, treatment decisions and treatment outcomes among blacks and non-blacks.Our study highlights possible racial differences between blacks and non-blacks, including a higher proportion of black women diagnosed with achalasia and most blacks presenting with dysphagia. There is possibly a meaningful interaction of race and sex in the development of achalasia that might represent genetic differences in its pathophysiology. Further prospective studies are required to identify such differences.