{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T04:07:21Z","timestamp":1762056441108,"version":"build-2065373602"},"reference-count":17,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2022,7,5]],"date-time":"2022-07-05T00:00:00Z","timestamp":1656979200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>(1) Background: Absent contractility (AC) is an esophageal motility disorder defined as a normal integrated relaxation pressure with 100% failed peristalsis. We sought to clarify the natural history of this disorder and its relationship with rheumatologic diseases, such as systemic sclerosis (scleroderma). (2) Methods: We retrospectively identified patients with AC based on high-resolution manometry findings at three referral institutions and then matched them with controls with esophageal complaints who had normal manometries. (3) Results: Seventy-four patients with AC were included (mean age 56 years; 69% female). Sixteen patients (21.6%) had a rheumatologic disease. Compared to controls, patients with AC were significantly more likely to present with heartburn, dysphagia, vomiting, and weight loss. During follow-up, they were also more likely to be seen by a gastroenterologist, be diagnosed with gastroesophageal reflux disease, take a proton pump inhibitor, and undergo repeat upper endoscopies. No AC patients developed a new rheumatologic disease during follow-up. No significant differences were noted in the clinical presentation or course of AC patients with rheumatologic disease compared to those without. (4) Conclusions: Patients with AC have more esophageal symptoms and require more intense gastrointestinal follow-up than controls. Only a minority of patients with AC have underlying rheumatologic disease. Those without rheumatologic disease at baseline did not subsequently develop one, suggesting that a rheumatologic evaluation is likely unnecessary. The clinical course of AC in patients with rheumatologic disease and those without appears to be similar.<\/jats:p>","DOI":"10.3390\/jcm11133922","type":"journal-article","created":{"date-parts":[[2022,7,5]],"date-time":"2022-07-05T10:21:33Z","timestamp":1657016493000},"page":"3922","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["The Natural History of Esophageal \u201cAbsent Contractility\u201d and Its Relationship with Rheumatologic Diseases: A Multi-Center Case\u2013Control Study"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7879-3966","authenticated-orcid":false,"given":"Daniel","family":"Cohen","sequence":"first","affiliation":[{"name":"The Gonczarowski Family Institute of Gastroenterology and Liver Diseases, Shamir (Assaf Harofeh) Medical Center, Zerifin 7030000, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6477-2849","authenticated-orcid":false,"given":"Ram","family":"Dickman","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Beilinson Hospital, Rabin Medical Center, Petach Tikva 4941492, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anton","family":"Bermont","sequence":"additional","affiliation":[{"name":"The Gonczarowski Family Institute of Gastroenterology and Liver Diseases, Shamir (Assaf Harofeh) Medical Center, Zerifin 7030000, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vered","family":"Richter","sequence":"additional","affiliation":[{"name":"The Gonczarowski Family Institute of Gastroenterology and Liver Diseases, Shamir (Assaf Harofeh) Medical Center, Zerifin 7030000, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haim","family":"Shirin","sequence":"additional","affiliation":[{"name":"The Gonczarowski Family Institute of Gastroenterology and Liver Diseases, Shamir (Assaf Harofeh) Medical Center, Zerifin 7030000, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amir","family":"Mari","sequence":"additional","affiliation":[{"name":"Gastroenterology and Endoscopy Unit, Nazareth EMMS Hospital, Nazareth 16100, Israel"},{"name":"Faculty of Medicine, Bar Ilan University, Safed 1311502, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1111\/nmo.12477","article-title":"The Chicago Classification of esophageal motility disorders, v3.0","volume":"27","author":"Kahrilas","year":"2015","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"e14058","DOI":"10.1111\/nmo.14058","article-title":"Esophageal motility disorders on high-resolution manometry: Chicago classification version 4.0\u00a9","volume":"33","author":"Yadlapati","year":"2021","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1136\/gut.49.1.145","article-title":"Classification of oesophageal motility abnormalities","volume":"49","author":"Spechler","year":"2001","journal-title":"Gut"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1093\/gastro\/goy018","article-title":"Gender, medication use and other factors associated with esophageal motility disorders in non-obstructive dysphagia","volume":"6","author":"Kamal","year":"2018","journal-title":"Gastroenterol. Rep."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1528","DOI":"10.1016\/j.jfma.2018.12.019","article-title":"Etiologies and clinical characteristics of non-obstructive dysphagia in a Taiwanese population: A prospective study based on high-resolution impedance manometry","volume":"118","author":"Yeh","year":"2019","journal-title":"J. Formos. Med. Assoc."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1111\/j.1442-2050.2010.01150.x","article-title":"Esophageal dysmotility associated with systemic sclerosis: A high-resolution manometry study","volume":"24","author":"Roman","year":"2011","journal-title":"Dis. Esophagus"},{"key":"ref_7","first-page":"165","article-title":"Evaluation and management of esophageal manifestations in systemic sclerosis","volume":"31","author":"Denaxas","year":"2018","journal-title":"Ann. Gastroenterol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1157","DOI":"10.1111\/nmo.12813","article-title":"The association between systemic sclerosis disease manifestations and esophageal high-resolution manometry parameters","volume":"28","author":"Kimmel","year":"2016","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1093\/dote\/dox067","article-title":"Spectrum of esophageal dysmotility in systemic sclerosis on high-resolution esophageal manometry as defined by Chicago classification","volume":"30","author":"Aggarwal","year":"2017","journal-title":"Dis. Esophagus"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"950","DOI":"10.1007\/s00535-019-01578-6","article-title":"Relationship between esophageal motility abnormalities and skin or lung involvements in patients with systemic sclerosis","volume":"54","author":"Kuribayashi","year":"2019","journal-title":"J. Gastroenterol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1007\/s10067-019-04794-w","article-title":"The role of high-resolution manometry in the assessment of upper gastrointestinal involvement in systemic sclerosis: A systematic review","volume":"39","author":"Schutyser","year":"2020","journal-title":"Clin. Rheumatol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1097\/MCG.0000000000000989","article-title":"Clinical Characteristics and Associated Systemic Diseases in Patients with Esophageal \u201cAbsent Contractility\u201d\u2014A Clinical Algorithm","volume":"53","author":"Laique","year":"2019","journal-title":"J. Clin. Gastroenterol."},{"key":"ref_13","first-page":"331","article-title":"Clinical spectrum and presentation of patients with absent contractility","volume":"34","author":"Kovacs","year":"2021","journal-title":"Ann. Gastroenterol."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Alcal\u00e1-Gonz\u00e1lez, L.G., Jimenez-Masip, A., Relea-P\u00e9rez, L., Barber-Caselles, C., and Barba-Orozco, E. (2022). Underlying etiology associated with the diagnosis of absent contractility on high resolution esophageal manometry. Gastroenterol. Hepatol.","DOI":"10.1016\/j.gastrohep.2022.03.003"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"796","DOI":"10.1111\/j.1365-2982.2009.01311.x","article-title":"High-resolution manometry in clinical practice: Utilizing pressure topography to classify oesophageal motility abnormalities","volume":"21","author":"Pandolfino","year":"2009","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1111\/j.1365-2982.2011.01834.x","article-title":"Chicago classification criteria of esophageal motility disorders defined in high resolution esophageal pressure topography1","volume":"24","author":"Bredenoord","year":"2012","journal-title":"Neurogastroenterol. Motil."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"848","DOI":"10.7326\/0003-4819-100-6-848","article-title":"Scleroderma Esophagus: A Nonspecific Entity","volume":"100","author":"Schneider","year":"1984","journal-title":"Ann. Intern. Med."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/13\/3922\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T23:43:05Z","timestamp":1760139785000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/11\/13\/3922"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,7,5]]},"references-count":17,"journal-issue":{"issue":"13","published-online":{"date-parts":[[2022,7]]}},"alternative-id":["jcm11133922"],"URL":"https:\/\/doi.org\/10.3390\/jcm11133922","relation":{},"ISSN":["2077-0383"],"issn-type":[{"type":"electronic","value":"2077-0383"}],"subject":[],"published":{"date-parts":[[2022,7,5]]}}}