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Disorders of esophageal motility are dened by using high resolu­tion manometry (HRM) with color pressure topography plots and categorized according to version 3.0 of the Chicago Classication of esophageal motility disorders, last updated in 2014 [1]. Using a tiered approach, the Chicago Classication system denes the primary esophageal motility disorders. In addition to those major and minor disorders, there are other systemic and localized pathologies which secondarily lead to disorders of motility. As a result of these secondary esophageal motility disorders being rel-

Workup can include pH testing with or without impedance and upper endoscopy, but manometry is imperative. Classic sclero­derma esophagus is characterized by absent or ineffective peri­stalsis with a hypotensive lower esophageal sphincter (LES). A hypotensive LES is dened as less than 10mmHg. Upwards of 50% of symptomatic patients will present with this manometric pattern. Additionally, chest CT may reveal a dilated esophagus in >70% of patients who present with the classic scleroderma esoph­agus pattern on esophageal manometry [3].

Management is aimed at symptom improvement, either man­aging GERD symptoms or dysmotility symptoms. GERD man­agement is centered on proton pump inhibitor (PPI) therapy, but although there is short term benet, the long-term efcacy is not sustained, and there is no prevention of progression of esophageal dysfunction [4, 5]. Common practice includes a 2–4× increase in the daily dose of PPI to gain better symptom control [6]. Surgical management for GERD symptoms of SSc should avoid anti-reux procedures such as Nissen fundoplication, as 38–71% of patients can develop postoperative dysphagia despite improvement of reux symptoms [7–9]. Some authors advocate Roux-en-y gastric bypass to prevent reux, which has been shown to have better post-op GER-related quality of life and less dysphagia as com­pared to Nissen [10]. However, bypass in a scleroderma patient should be pursued with caution given the propensity of small intestinal dysmotility in SSc patients. An esophagectomy can be considered for end-stage scleroderma of the esophagus.

Transient LES relaxations are 10–60s periods of LES relax­ation that are not preceded by a swallow and are deemed sponta­neous [20]. These episodes make up the majority of the periods of reux in patients with moderate GERD.As the severity of GERD increases, a hypotensive LES becomes the prevalent pathophysiological mechanism. Dened as an LES basal pressure less than 10mmHg, a hypotensive LES is found with increasing prevalence as the severity of GERD increases, such as in patients with Barrett’s esophagus, erosive esophagitis, and nonerosive reux disease [21, 22].

Diagnosis of esophageal dysmotility in the setting of GERD is done with high resolution manometry, often combined with impedance, which can help identify abnormal bolus transport and clearance during swallows and investigate the relationships between bolus transit and LES relaxation [28]. Treatment with a proton pump inhibitor will decrease acid exposure, but does not improve esophageal dysmotility. Additionally, weight loss will