“…These include a high prevalence of GERD (66%-100%), 8,37,106,107 45% rate of esophagitis, 8 13% rate of Barrett's esophagus, 76 a high frequency of ARP (31%-65%), 8,37,106,107 and a high prevalence of AS (57%-79%). 8,37,76,93 These high-risk patients generally receive long-term PPI treatments. 106 However, due to the heterogeneity of treatment in this rare form of EA, almost no data exist on PPI efficacy; a single retrospective study reported GERD symptom improvement in 69% of cases.…”