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Marten Canavesio Y, Pasta A, Calabrese F, Alessandri E, Cutolo M, Paolino S, Pizzorni C, Sulli A, Savarino V, Giannini EG, Zentilin P, Bodini G, Furnari M, Savarino E, Marabotto E. Association between esophageal motor disorders and pulmonary involvement in patients affected by systemic sclerosis: a retrospective study. Rheumatol Int 2024; 44:2905-2910. [PMID: 37542603 DOI: 10.1007/s00296-023-05399-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/14/2023] [Accepted: 07/19/2023] [Indexed: 08/07/2023]
Abstract
Systemic sclerosis (SSc) is a rare autoimmune disease of the connective tissue that can affect multiple organs. The esophagus is the most affected gastrointestinal tract, while interstitial lung disease (ILD) is a main feature associated with SSc. The aim of the present study was to evaluate the association and prognostic implication between motor esophageal disorders and pulmonary involvement in SSc patients. We retrospectively assessed patients with SSc who underwent both the HRM with the new Chicago Classification 4.0 and pulmonary evaluation comprehensive of function tests and high-resolution computer tomography (HrCT) with the use of Warrick score. A total score ≥ 7 was considered predictive of ILD, while a score ≥ 10 in a HrCT acquired prospectively from baseline evaluation was considered to establish significant interstitial involvement. Forty-two patients were included. We found a score ≥ 7 in 11 patients with aperistalsis, in 6 subjects with IEM and in 6 patients with a normal manometry. Otherwise, a score < 7 was observed in 3 patients with aperistalsis, and in 2 and 14 patients with IEM and with a normal contractility, respectively. Higher scores were observed in subjects with absent contractility or ineffective esophageal motility than subjects with normal motility, indeed DCI and HrCT score were inversely correlated in linear and logarithmic regression analysis. Prospectively, lower baseline LESP and greater HrCT scores at follow-up evaluation were significantly correlated. This study shows an association between motor esophageal disorder and pulmonary involvement in SSc patients: more severe is the esophageal involvement, more critical is the pulmonary disease.
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Affiliation(s)
- Ylenia Marten Canavesio
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS-Ospedale Policlinico San Martino, 16132, Genoa, Italy
| | - Andrea Pasta
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS-Ospedale Policlinico San Martino, 16132, Genoa, Italy.
| | - Francesco Calabrese
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS-Ospedale Policlinico San Martino, 16132, Genoa, Italy
| | - Elisa Alessandri
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, Genoa, Italy
| | - Maurizio Cutolo
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, Genoa, Italy
| | - Sabrina Paolino
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, Genoa, Italy
| | - Carmen Pizzorni
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, Genoa, Italy
| | - Alberto Sulli
- Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine DiMI, University of Genoa, IRCCS San Martino Polyclinic, Genoa, Italy
| | - Vincenzo Savarino
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS-Ospedale Policlinico San Martino, 16132, Genoa, Italy
| | - Edoardo Giovanni Giannini
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS-Ospedale Policlinico San Martino, 16132, Genoa, Italy
| | - Patrizia Zentilin
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS-Ospedale Policlinico San Martino, 16132, Genoa, Italy
| | - Giorgia Bodini
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS-Ospedale Policlinico San Martino, 16132, Genoa, Italy
| | - Manuele Furnari
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS-Ospedale Policlinico San Martino, 16132, Genoa, Italy
| | - Edoardo Savarino
- Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy
- Gastroenterology Unit, Azienda Ospedale Università di Padua, Padua, Italy
| | - Elisa Marabotto
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, IRCCS-Ospedale Policlinico San Martino, 16132, Genoa, Italy
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Hoshino S, Momma E, Koeda M, Hoshikawa Y, Tanabe T, Kawami N, Kitasako Y, Iwakiri K. In females, salivary secretion was significantly lower in patients with severe reflux esophagitis than in healthy controls. Esophagus 2024; 21:383-389. [PMID: 38536601 DOI: 10.1007/s10388-024-01052-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/31/2023] [Accepted: 02/19/2024] [Indexed: 06/27/2024]
Abstract
BACKGROUND The salivary secretion in patients with mild reflux esophagitis (RE) and non-erosive reflux disease is significantly lower in females, but not in males. However, sex differences in salivary secretion in patients with severe RE remain unknown. Therefore, the present study investigated sex differences in saliva secretion in patients with severe RE. METHODS Subjects consisted of 23 male patients with severe RE, 24 male healthy controls (HCs), 26 female patients with severe RE, and 25 female HCs. Saliva secretion was assessed as follows: each patient chewed sugarless gum for 3 min prior to endoscopy, and the amount and pH of saliva secreted before and after acid loading as an index of the acid-buffering capacity were measured. RESULTS In males, no significant differences were observed in the amount of saliva secretion, salivary pH, or the acid-buffering capacity between severe RE patients and HCs. In females, the amount of saliva secretion (severe RE: 2.4 [1.8-4.1], HCs: 5.3 [3.4-7.5], p = 0.0017), salivary pH (severe RE: 7.0 [6.7-7.3], HCs: 7.2 [7.1-7.3], p = 0.0455), and the acid-buffering capacity (severe RE: 5.9 [5.3-6.2], HCs: 6.2 [6.1-6.5], p = 0.0024) were significantly lower in severe RE patients than in HCs. CONCLUSION Among females, the salivary secretion was significantly lower in severe RE patients than in HCs. This reduction in salivary secretion may contribute to the pathophysiology of severe RE in females.
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Affiliation(s)
- Shintaro Hoshino
- Department of Gastroenterology, Nippon Medical School, Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan
| | - Eri Momma
- Department of Gastroenterology, Nippon Medical School, Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan
| | - Mai Koeda
- Department of Gastroenterology, Nippon Medical School, Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan
| | - Yoshimasa Hoshikawa
- Department of Gastroenterology, Nippon Medical School, Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan
| | - Tomohide Tanabe
- Department of Gastroenterology, Nippon Medical School, Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan
| | - Noriyuki Kawami
- Department of Gastroenterology, Nippon Medical School, Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan
| | - Yuichi Kitasako
- Department of Gastroenterology, Nippon Medical School, Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan
- Cariology and Operative Dentistry, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Bunkyo-Ku, Tokyo, Japan
- Dental Clinic, Ministry of Foreign Affairs, Chiyoda-Ku, Tokyo, Japan
| | - Katsuhiko Iwakiri
- Department of Gastroenterology, Nippon Medical School, Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan.
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Kuribayashi S, Nakamura F, Motegi SI, Hara K, Hosaka H, Sekiguchi A, Ishikawa M, Endo Y, Harada T, Sorimachi H, Obokata M, Uchida M, Yamaguchi K, Uraoka T. Prevalence and risk factors for medication-refractory reflux esophagitis in patients with systemic sclerosis in Japan. J Gastroenterol 2024; 59:179-186. [PMID: 38252140 DOI: 10.1007/s00535-024-02076-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/24/2023] [Accepted: 01/02/2024] [Indexed: 01/23/2024]
Abstract
BACKGROUNDS Patients with systemic sclerosis (SSc) often have esophageal motility abnormalities and weak esophago-gastric junction (EGJ) barrier function, which causes proton pump inhibitor (PPI)-refractory reflux esophagitis (RE). The aims of this study were to clarify the current management of RE and prevalence and risk factors of medication-refractory RE in patients with SSc in Japan. METHODS A total of 188 consecutive patients with SSc who underwent both esophageal high-resolution manometry (HRM) and esophagogastroduodenoscopy (EGD) were reviewed. The presence of RE and grades of the gastroesophageal flap valve (GEFV) were assessed. Esophageal motility was assessed retrospectively according to the Chicago classification v3.0. When RE was seen on a standard dose of PPI or any dose of vonoprazan (VPZ), it was defined as medication-refractory RE. RESULTS Approximately 80% of patients received maintenance therapy with acid secretion inhibitors regardless of esophageal motility abnormalities. Approximately 50% of patients received maintenance therapy with PPI, and approximately 30% of patients received VPZ. Medication-refractory RE was observed in 30 patients (16.0%). In multivariable analyses, the number of EGD and absent contractility were significant risk factors for medication-refractory RE. Furthermore, combined absent contractility and GEFV grade III or IV had higher odds ratios than did absent contractility alone. CONCLUSIONS Patients with persistent reflux symptoms and those with absent contractility and GEFV grade III or IV should receive maintenance therapy with strong acid inhibition to prevent medication-refractory RE.
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Affiliation(s)
- Shiko Kuribayashi
- Department of Gastroenterology and Hepatology, Gunma University Graduate School of Medicine, 3-39-15 Showa-Machi Maebashi, Gunma, 371-8511, Japan.
| | - Fumihiko Nakamura
- Department of Gastroenterology and Hepatology, Gunma University Graduate School of Medicine, 3-39-15 Showa-Machi Maebashi, Gunma, 371-8511, Japan
| | - Sei-Ichiro Motegi
- Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Kenichiro Hara
- Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Hiroko Hosaka
- Department of Gastroenterology and Hepatology, Gunma University Graduate School of Medicine, 3-39-15 Showa-Machi Maebashi, Gunma, 371-8511, Japan
| | - Akiko Sekiguchi
- Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Mai Ishikawa
- Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Yukie Endo
- Department of Dermatology, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Tomonari Harada
- Department of Cardiovascular Medicine, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Hidemi Sorimachi
- Department of Cardiovascular Medicine, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Masaru Obokata
- Department of Cardiovascular Medicine, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Mitsuo Uchida
- Department of Public Health, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Koichi Yamaguchi
- Department of Allergy and Respiratory Medicine, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan
| | - Toshio Uraoka
- Department of Gastroenterology and Hepatology, Gunma University Graduate School of Medicine, 3-39-15 Showa-Machi Maebashi, Gunma, 371-8511, Japan
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Clarke JO, Ahuja NK. Upper Gastrointestinal Tract: Manifestations of Systemic Sclerosis. SCLERODERMA 2024:477-493. [DOI: 10.1007/978-3-031-40658-4_31] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Marabotto E, Savarino V, Savarino E. Towards a more precise classification of esophageal motility disorders in patients with systemic sclerosis. Neurogastroenterol Motil 2022; 34:e14416. [PMID: 35593267 DOI: 10.1111/nmo.14416] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/05/2022] [Revised: 04/21/2022] [Accepted: 05/04/2022] [Indexed: 02/08/2023]
Abstract
Systemic sclerosis (SSc) is a chronic and generalized disease affecting the connective tissue of the skin and many internal organs, in particular the gastrointestinal tract. The esophagus is involved in up to 80% of the cases and represents a major cause of serious morbidities that deeply impact on the quality of life and survival of patients. Indeed, the presence of esophageal dysfunction is a good prognostic indicator in SSc, primarily due to its impact on pulmonary disease. Thus, the detection of esophageal motility alterations plays a critical role to prevent the development of both esophageal and pulmonary complications and to improve the survival of these patients. Currently, this diagnostic work-up has been limited to the use of esophageal manometry, which is considered the gold standard for the evaluation of motor physiology and pathophysiology of this organ in different clinical situations. However, in recent years, new equipments such as high-resolution (-impedance) manometry and functional luminal imaging probe have been developed and used in many esophageal clinical settings, including SSc. In this mini-review, we summarize current evidence regarding esophageal dysmotility, in the light of new data on secondary peristalsis published in this issue of the journal.
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Affiliation(s)
- Elisa Marabotto
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, Genoa, Italy
| | - Vincenzo Savarino
- Gastroenterology Unit, Department of Internal Medicine, University of Genoa, Genoa, Italy
| | - Edoardo Savarino
- Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy.,Gastroenterology Unit, Azienda Ospedale Università di Padova, Padua, Italy
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Abstract
The gastrointestinal tract is the second largest organ system in the body and is often affected by connective tissue disorders. Scleroderma is the classic rheumatologic disease affecting the esophagus; more than 90% of patients with scleroderma have esophageal involvement. This article highlights esophageal manifestations of scleroderma, focusing on pathogenesis, clinical presentation, diagnostic considerations, and treatment options. In addition, this article briefly reviews the esophageal manifestations of other key connective tissue disorders, including mixed connective tissue disease, myositis, Sjogren syndrome, systemic lupus erythematosus, fibromyalgia, and Ehlers-Danlos syndrome.
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Affiliation(s)
- Nitin K Ahuja
- Division of Gastroenterology and Hepatology, University of Pennsylvania, 3400 Civic Center Boulevard 7 South Pavilion, Philadelphia, PA 19104, USA
| | - John O Clarke
- Division of Gastroenterology and Hepatology, Stanford University School of Medicine, 430 Broadway Street, Pavilion C, 3rd Floor, C-343, Redwood City, CA 94063-6341, USA.
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Lee JS, Kim HS, Moon JR, Ryu T, Hong SJ, Cho YS, Park J, Lee TH. Esophageal Involvement and Determinants of Perception of Esophageal Symptoms Among South Koreans With Systemic Sclerosis. J Neurogastroenterol Motil 2020; 26:477-485. [PMID: 32989185 PMCID: PMC7547196 DOI: 10.5056/jnm19148] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/06/2019] [Revised: 03/09/2020] [Accepted: 07/03/2020] [Indexed: 12/11/2022] Open
Abstract
Background/Aims Our study aims to characterize esophageal motor function; evaluate the relationships among esophagogastroduodenoscopy (EGD), high-resolution manometry (HRM), and 24-hour esophageal multichannel intraluminal impedance monitoring combined with pH-metry (MII-pH); and elucidate the determinants of esophageal symptom perception in South Koreans with systemic sclerosis (SSc). Methods We reviewed prospectively collected HRM (n = 46), EGD (n = 41), and MII-pH (n = 37) data from 46 consecutive patients with SSc (42 females; mean age 50.1 years) who underwent esophageal tests between June 2013 and September 2018. Results The most common HRM diagnosis was normal (39.1%), followed by ineffective esophageal motility (23.9%) and absent contractility (21.7%). Erosive esophagitis was observed in 12.2% of total SSc patients, with a higher frequency in patients with absent contractility than those with normal motility (44.5% vs 0.0%, P = 0.01). Pathologic acid exposure was observed in 6 patients (20.0%) and positive symptom association in 18 patients (60.0%) in MII-pH tests of symptomatic patients. The proportion of SSc patients with esophageal symptoms not explained by reflux or mucosal or motor esophageal abnormalities was 33.0%. Conclusions Esophageal involvement among South Koreans with SSc was characterized by heterogeneous motility patterns, with a higher prevalence of normal motility and lower prevalence of erosive esophagitis. Reflux hypersensitivity or functional heartburn might be partly attributed to the perception of esophageal symptoms in SSc patients who have neither gastroesophageal reflux disease nor esophageal dysmotility.
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Affiliation(s)
- Joon Seong Lee
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Hyun-Sook Kim
- Department of Rheumatology, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Jung Rock Moon
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Tom Ryu
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Su Jin Hong
- Department of Internal Medicine, Soonchunhyang University College of Medicine, Bucheon, Gyeonggi-do, Korea
| | - Young Sin Cho
- Division of Gastroenterology, Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan, Chungcheongnam-do, Korea
| | - Junseok Park
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
| | - Tae Hee Lee
- Institute for Digestive Research, Digestive Disease Center, Soonchunhyang University College of Medicine, Seoul, Korea
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Nicola S, Rolla G, Bucca C, Geronazzo G, Ridolfi I, Ferraris A, Fusaro E, Peroni CL, Dughera L, Brussino L. Gastric Juice Expression of Th-17 and T-Reg Related Cytokines in Scleroderma Esophageal Involvement. Cells 2020; 9:E2106. [PMID: 32947843 PMCID: PMC7564480 DOI: 10.3390/cells9092106] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/05/2020] [Revised: 09/07/2020] [Accepted: 09/11/2020] [Indexed: 01/26/2023] Open
Abstract
BACKGROUND Systemic sclerosis (SSc) is a connective tissue disorder which key feature is a fibrotic process. The role of Endothelin-1 (ET-1) and T-helper (Th)-1 cells in lung and skin fibrosis is well known, although Th17- and Treg-cells were found to be involved. However, no studies analyzed cytokines expression in gastric-juice of SSc patients. Our study aimed to evaluate proinflammatory and profibrotic cytokines in gastric-juice of SSc patients and to investigate their correlations with esophageal dysmotility. METHODS Patients performed upper-gastrointestinal-endoscopy with gastric-juice collection, esophageal manometry and thoracic CT-scan. GM-CSF, ET-1, Th-1 (IFN-γ, IL-1β, TNF-α, IL-2, IL-6, IL-9), Th-17 (IL-17, IL-21, IL-22, IL-23) and T-reg (IL-10, TGF-β) related cytokines were measured in 29 SSc-patients and 20 healthy-controls. RESULTS Patients showed significant lower levels of IL-6, IL-17, IL-22 and ET-1 (p < 0.005) compared with controls. Patients with atrophic gastritis presented significant lower levels of IL-2, IL-9, IL-6, TGF-β, GM-CSF, IL-17 and ET-1 (p < 0.005) compared to patients without gastritis. Increased values of IL-2, IL-9, IL-1β, IL-17, ET-1 and GM-CSF (p < 0.005) were observed in patients with esophageal impairment. This is the first report of cytokines measurement in gastric juice of patients with SSc. The high IL-17 concentrations in gastric-juice of scleroderma patients with esophageal dysmotility support the signature of Th-17 cells in scleroderma esophageal fibrosis.
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Affiliation(s)
- Stefania Nicola
- Department of Medical Sciences, Allergy and Clinical Immunology Unit, University of Torino & Mauriziano Hospital, 10128 Turin, Italy; (S.N.); (G.R.); (C.B.); (G.G.); (I.R.)
| | - Giovanni Rolla
- Department of Medical Sciences, Allergy and Clinical Immunology Unit, University of Torino & Mauriziano Hospital, 10128 Turin, Italy; (S.N.); (G.R.); (C.B.); (G.G.); (I.R.)
| | - Caterina Bucca
- Department of Medical Sciences, Allergy and Clinical Immunology Unit, University of Torino & Mauriziano Hospital, 10128 Turin, Italy; (S.N.); (G.R.); (C.B.); (G.G.); (I.R.)
| | - Giada Geronazzo
- Department of Medical Sciences, Allergy and Clinical Immunology Unit, University of Torino & Mauriziano Hospital, 10128 Turin, Italy; (S.N.); (G.R.); (C.B.); (G.G.); (I.R.)
| | - Irene Ridolfi
- Department of Medical Sciences, Allergy and Clinical Immunology Unit, University of Torino & Mauriziano Hospital, 10128 Turin, Italy; (S.N.); (G.R.); (C.B.); (G.G.); (I.R.)
| | - Andrea Ferraris
- Division of Diagnostic Imaging, Department of Surgical Sciences, Città della Salute e della Scienza Hospital, University of Turin, 10126 Turin, Italy;
| | - Enrico Fusaro
- Rheumatology Department, Azienda Ospedaliera Città della Salute e della Scienza di Torino, 10126 Turin, Italy; (E.F.); (C.L.P.)
| | - Clara Lisa Peroni
- Rheumatology Department, Azienda Ospedaliera Città della Salute e della Scienza di Torino, 10126 Turin, Italy; (E.F.); (C.L.P.)
| | - Luca Dughera
- Unit of Digestive Motility and Endoscopy, Department of Medicine, Città della Salute e della Scienza, 10126 Turin, Italy;
| | - Luisa Brussino
- Department of Medical Sciences, Allergy and Clinical Immunology Unit, University of Torino & Mauriziano Hospital, 10128 Turin, Italy; (S.N.); (G.R.); (C.B.); (G.G.); (I.R.)
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Petcu A, Ghib LJ, Grad SM, Popovici C, Rogojan L, Rednic NV, Rednic S. Upper gastrointestinal involvement in systemic sclerosis: Findings in a real-life setting. Exp Ther Med 2019; 18:5095-5100. [PMID: 31798729 PMCID: PMC6880395 DOI: 10.3892/etm.2019.8125] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/30/2019] [Accepted: 10/01/2019] [Indexed: 12/21/2022] Open
Abstract
Gastrointestinal tract involvement is the most common visceral affectation in systemic sclerosis (SSc), but the manifestations may vary in extension and severity. Endoscopic and histopathological gastroesophageal findings were investigated in patients with SSc. A total of 79 consecutive patients with definite SSc were enrolled in a cross sectional study. Clinical data were collected, upper gastrointestinal endoscopy and biopsies from gastric mucosa were performed in all cases. Fifty-seven (72.1%) out of 79 SSc patients had gastroesophageal symptoms. The most frequent were dysphagia, present in 33 (41.7%) and gastroesophageal reflux symptoms in 23 (29.1%) patients. Out of the 79 patients, 22 were asymptomatic, but in 16 esophageal and gastric mucosa changes were endoscopically detected. Reflux esophagitis was found in 39 (49.3%) patients. The presence of esophageal manifestations was not related to the disease duration or with its other variables. Signs of gastritis were endoscopically described in 47 (59.4%) and confirmed on histopathologic examinations in 45 patients. In 31 patients without any endoscopic changes, 18 (22.7%) showed signs of gastritis on histopathologic examination. No significant statistical differences were found between symptomatic and asymptomatic patients or between those with limited cutaneous SSc and those with diffuse cutaneous SSc in terms of clinical, endoscopic or histopathological findings, except the higher proportion of hiatal hernia in symptomatic patients. The results of this study might suggest that upper gastrointestinal endoscopy should be performed during the early stage of the disease and then periodically in patients diagnosed with SSc, even in the absence of typical symptoms.
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Affiliation(s)
- Ana Petcu
- Rheumatology Department, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania
| | - Linda Jessica Ghib
- Rheumatology Department, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania
| | - Simona Mihaela Grad
- 2nd Medical Clinic, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania
| | - Cornelia Popovici
- 1st Medical Clinic, Gastroenterology Department, Emergency Clinical County Hospital, 400006 Cluj-Napoca, Romania
| | - Liliana Rogojan
- Department of Pathology, Emergency Clinical County Hospital, 400006 Cluj-Napoca, Romania
| | - Nicolae Voicu Rednic
- 3rd Medical Clinic, Gastroenterology Department, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400162 Cluj-Napoca, Romania
| | - Simona Rednic
- Rheumatology Department, ‘Iuliu Haţieganu’ University of Medicine and Pharmacy, 400006 Cluj-Napoca, Romania
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Ostojic P, Jankovic K, Djurovic N, Stojic B, Knezevic-Apostolski S, Bartolovic D. Common Causes of Pain in Systemic Sclerosis: Frequency, Severity, and Relationship to Disease Status, Depression, and Quality of Life. Pain Manag Nurs 2019; 20:331-336. [PMID: 31103501 DOI: 10.1016/j.pmn.2019.02.006] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2018] [Revised: 02/07/2019] [Accepted: 02/13/2019] [Indexed: 11/22/2022]
Abstract
BACKGROUND In routine clinical practice, healthcare professionals draw little attention to pain in patients with systemic sclerosis (SSc). Pain has adverse effects on functional ability, social and emotional wellbeing. AIMS This study aims to assess the frequency and severity of different types of pain in patients with SSc, and the relationship of pain with disease status, depression and quality of life. DESIGN Consecutive patients with SSc were included in this cross-sectional study. Patients with previously diagnosed painful diseases or conditions (other rheumatic diseases, angina pectoris, neurological disorders, etc.) were excluded. SETTINGS Patients, who visited our rheumatology outpatient clinic from February to November 2016, participated in this study. PARTICIPANTS/SUBJECTS 42 consecutive patients with SSc (38 women and 4 men), mean age 56.5 years, mean disease duration 9.5 years, were included. METHODS All patients filled in a questionnaire, to indicate the presence or absence of some predefined pain syndromes. Disease status was assessed using the Scleroderma Assessment Questionnaire (SAQ), symptoms of depression by the Beck's Depression Inventory (BDI), whilst the quality of life was evaluated using the EuroQol questionnaire. RESULTS It was found that 92.9% of SSc patients suffer from different types of pain, and 45.2% of patients have pain every day. Joint pain was the most common type of pain, present in 78.6% of patients, followed by pain associated with Raynaud's phenomenon (69%), back pain (47.6%), headache (31%), chest pain (23.8%), odynophagia (21.4%) and painful digital ulcers (19%). Symptoms of neuropathic pain were noticed in 26.2% of patients. Severe joint pain, everyday pain and symptoms of neuropathic pain in SSc were associated with more severe disease and poorer quality of life. Pain related to Raynaud's phenomenon, digital ulcers, odynophagia and joint pain were associated with significant symptoms of depression. CONCLUSION The majority of patients with SSc suffer from different types of pain. Pain is associated with more severe disease, depression and poor quality of life.
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Affiliation(s)
- Predrag Ostojic
- Institute of Rheumatology, Belgrade, Serbia; School of Medicine, University of Belgrade, Serbia.
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11
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Tétreault MP, Kahrilas P. GI Manifestations With a Focus on the Esophagus: Recent Progress in Understanding Pathogenesis. Curr Rheumatol Rep 2019; 21:42. [PMID: 31270707 DOI: 10.1007/s11926-019-0841-x] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
PURPOSE OF REVIEW Esophageal dysfunction is common in systemic sclerosis (SSc) patients. Limited treatment options are available for scleroderma esophageal disease. Here, we discuss recent updates on the diagnosis, treatment, and characterization that have been made in patients with scleroderma esophageal disease. RECENT FINDINGS In the past few years, novel diagnostic tools have provided insight into esophageal dysmotility in SSc patients. New drugs are being tested and might improve symptoms and quality of life in SSc patients with esophageal dysfunction. Molecular stratification methods have facilitated the identification of molecular signatures in the esophagus of SSc patients. The Friend leukemia integration 1 (Fli1) conditional knockout mouse is the first animal model to report an esophageal phenotype with SSc features. The clinical presentation in SSc patients with esophageal dysfunction is heterogeneous, complicating diagnosis and management. The improvement of diagnostic tools for esophageal symptoms and dysfunction and the use of molecular approaches in SSc mouse models and patient biopsies offer an opportunity to improve the characterization of SSc esophageal disease, which should help improve management and treatment decisions.
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Affiliation(s)
- Marie-Pier Tétreault
- Department of Medicine, Gastroenterology and Hepatology Division, Northwestern University Feinberg School of Medicine, 15-753 Tarry Building, 300 East Superior Street, Chicago, IL, 60611-3010, USA.
| | - Peter Kahrilas
- Department of Medicine, Gastroenterology and Hepatology Division, Northwestern University Feinberg School of Medicine, 15-753 Tarry Building, 300 East Superior Street, Chicago, IL, 60611-3010, USA
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12
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Bütikofer S, Jordan S, Sauter M, Hollenstein M, Heinrich H, Freitas-Queiroz N, Kuntzen T, Ang D, Oberacher M, Maurer B, Schwizer W, Fox M, Distler O, Misselwitz B. Abnormal esophageal motility during a solid test meal in systemic sclerosis-detection even in very early disease and association with disease progression. Neurogastroenterol Motil 2019; 31:e13480. [PMID: 30276930 DOI: 10.1111/nmo.13480] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/05/2018] [Revised: 08/10/2018] [Accepted: 09/03/2018] [Indexed: 02/08/2023]
Abstract
OBJECTIVE This study assessed whether high-resolution manometry (HRM) with a test meal can detect clinically relevant, abnormal motility already in very early systemic sclerosis (SSc) and whether this finding is associated with subsequent disease progression. METHODS This prospective, longitudinal cohort study recruited 68 consecutive SSc patients (group #1: 32 established disease (ACR, American College of Rheumatology /EULAR, The European League against Rheumatism 2013 and ACR 1980 criteria fulfilled); group #2: 24 early disease (only ACR/EULAR 2013 fulfilled); group #3: 12 very early disease (clinical expert diagnosis of SSc) and 72 healthy controls. HRM evaluated esophageal motility for water swallows and a solid test meal. RESULTS Systemic sclerosis patients had less frequent effective esophageal contractions during the test meal compared to healthy controls even in very early disease (0.15, 1.0, 2.1 per minute for groups #1, #2, and #3, vs 2.5 per minute in health; P < 0.001, P < 0.001, and P < 0.0085, respectively). Ineffective motility at HRM was associated with a higher modified Rodnan skin score at baseline. Moreover, at mean 18 (10-31) months of follow-up, the presence of ineffective motility at baseline was associated with progression of skin disease (P = 0.01). Cox proportional hazard regression analysis identified hypotensive peristalsis in the test meal (<15% effective solid swallows) and low distal contractile integral (DCI; <400 mm Hg·cm·s) as predictors for skin aggravation, but not for new organ involvement. CONCLUSION Ineffective motility during a test meal is present already in patients with very early SSc. Findings on HRM studies are associated with disease severity at baseline, and low percentage of effective swallows in test meal and low mean DCI are both predictors of skin progression during follow-up.
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Affiliation(s)
- Simon Bütikofer
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
| | - Suzana Jordan
- Department of Rheumatology, University Hospital Zurich and Zurich University, Zurich, Switzerland
| | - Matthias Sauter
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland.,Abdominal Center Gastroenterology, Basel, Switzerland
| | - Michael Hollenstein
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
| | - Henriette Heinrich
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland.,Abdominal Center Gastroenterology, Basel, Switzerland
| | - Natália Freitas-Queiroz
- Department of Gastroenterology, School of Medicine, University of São Paulo, São Paulo, Brazil
| | - Thomas Kuntzen
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
| | - Daphne Ang
- Department of Gastroenterology, Changi General Hospital, Singapore
| | - Marcos Oberacher
- Department of Medicine, Spital Limmattal, Schlieren, Switzerland
| | - Britta Maurer
- Department of Rheumatology, University Hospital Zurich and Zurich University, Zurich, Switzerland
| | - Werner Schwizer
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
| | - Mark Fox
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland.,Abdominal Center Gastroenterology, Basel, Switzerland
| | - Oliver Distler
- Department of Rheumatology, University Hospital Zurich and Zurich University, Zurich, Switzerland
| | - Benjamin Misselwitz
- Department of Gastroenterology and Hepatology, University Hospital Zurich and Zurich University Switzerland, Zurich, Switzerland
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13
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Arif T, Adil M, Singh Sodhi J, Hassan I. Assessment of modified Rodnan skin score and esophageal manometry in systemic sclerosis: a study correlating severity of skin and esophageal involvement by objective measures. ACTA DERMATOVENEROLOGICA ALPINA PANNONICA ET ADRIATICA 2018. [DOI: 10.15570/actaapa.2018.36] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
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14
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Abstract
PURPOSE OF REVIEW The purpose of this study was to highlight recent findings on evaluation for lung transplantation and outcomes after transplantation in patients with systemic sclerosis (scleroderma). RECENT FINDINGS The recognition that extra-pulmonary disease manifestations can seriously compromise post-transplant outcomes has changed the way patients are screened for lung transplant candidacy. Reluctance to transplant subjects affected by scleroderma has been driven by the complexity and multisystem nature of this disease. Multiple recent reports provide convincing findings that scleroderma patients undergoing lung transplantation have similar outcomes as those with other non-rheumatologic pulmonary conditions, even when significant esophageal dysmotility is present. New evidence supports the notion that scleroderma should not be a contraindication for referral to lung transplant. Future studies are needed to improve risk stratification, to define protocols for screening and management of extra-pulmonary complications, and to optimize immunosuppression before and after transplant.
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15
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Furst DE, Braun-Moscovic Y, Khanna D. Points to consider for clinical trials of the gastrointestinal tract in systemic sclerosis. Rheumatology (Oxford) 2017; 56:v4-v11. [PMID: 28992166 DOI: 10.1093/rheumatology/kex195] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2015] [Indexed: 12/19/2022] Open
Abstract
The pathogenesis of gastrointestinal tract involvement in SSc is not fully understood. However, gastrointestinal signs and symptoms are very common. Trials to test therapies, with rare exceptions, should be double-blind, randomized trials with either active therapy or placebo as comparators. Trial duration will vary dependent on the anticipated therapy and should usually be 6-24 weeks long, although some motility trials may need to be 52 weeks. As in any well-controlled trial, inclusion and exclusion criteria should encourage relatively uniform patients with sufficiently active disease to discern response, importantly considering disease duration. Previous therapy, co-morbid conditions, potentially confounding and/or concomitant therapy should be considered. Outcome measures should include both objective/semi-objective and subjective measures, although validated measures are not frequent and design needs to consider using only validated measures. Unvalidated measures can be included to validate them for future use. A full analysis plan should be completed before study commencement, including the method to account for missing data.
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Affiliation(s)
- Daniel E Furst
- Department of Rheumatology, Geffen School of Medicine, University of California, Los Angeles, CA, USA
| | - Yolanda Braun-Moscovic
- B.Shine Rheumatology Unit, Rambam Medical Health Care Campus, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technology, Haifa, Israel.,Rappaport Faculty of Medicine, Technion-Institute of Technology, Haifa, Israel
| | - Dinesh Khanna
- Division of Rheumatology, University of Michigan, Ann Arbor, MI, USA
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16
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Di Piazza A, Vernuccio F, Costanzo M, Scopelliti L, Picone D, Midiri F, Salvaggi F, Cupido F, Galia M, Salerno S, Lo Casto A, Midiri M, Lo Re G, Lagalla R. The Videofluorographic Swallowing Study in Rheumatologic Diseases: A Comprehensive Review. Gastroenterol Res Pract 2017; 2017:7659273. [PMID: 28706536 PMCID: PMC5494561 DOI: 10.1155/2017/7659273] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/18/2016] [Accepted: 04/11/2017] [Indexed: 02/01/2023] Open
Abstract
Autoimmune connective tissue diseases are a heterogeneous group of pathologies that affect about 10% of world population with chronic evolution in 20%-80%. Inflammation in autoimmune diseases may lead to serious damage to other organs including the gastrointestinal tract. Gastrointestinal tract involvement in these patients may also due to both a direct action of antibodies against organs and pharmacological therapies. Dysphagia is one of the most important symptom, and it is caused by failure of the swallowing function and may lead to aspiration pneumonia, malnutrition, dehydration, weight loss, and airway obstruction. The videofluorographic swallowing study is a key diagnostic tool in the detection of swallowing disorders, allowing to make an early diagnosis and to reduce the risk of gastrointestinal and pulmonary complications. This technique helps to identify both functional and structural anomalies of the anatomic chain involved in swallowing function. The aim of this review is to systematically analyze the basis of the pathological involvement of the swallowing function for each rheumatological disease and to show the main features of the videofluorographic study that may be encountered in these patients.
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Affiliation(s)
- Ambra Di Piazza
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | | | - Massimo Costanzo
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | - Laura Scopelliti
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | - Dario Picone
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | - Federico Midiri
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | - Francesco Salvaggi
- Unit of Colorectal Surgery, Department of Medical, Surgical, Neurological, Metabolic and Ageing Sciences, Second University of Naples, Naples, Italy
| | - Francesco Cupido
- Department of Surgical, Oncologic and Stomatologic Diseases, University of Palermo, Palermo, Italy
| | - Massimo Galia
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | - Sergio Salerno
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | - Antonio Lo Casto
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | - Massimo Midiri
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | - Giuseppe Lo Re
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
| | - Roberto Lagalla
- Section of Radiology-Di.Bi.Med., University of Palermo, Palermo, Italy
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17
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Kimmel JN, Carlson DA, Hinchcliff M, Carns MA, Aren KA, Lee J, Pandolfino JE. The association between systemic sclerosis disease manifestations and esophageal high-resolution manometry parameters. Neurogastroenterol Motil 2016; 28:1157-65. [PMID: 26921101 PMCID: PMC4956560 DOI: 10.1111/nmo.12813] [Citation(s) in RCA: 57] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/14/2015] [Accepted: 02/03/2016] [Indexed: 12/15/2022]
Abstract
BACKGROUND We aimed to evaluate the associations between systemic sclerosis (SSc)-related systemic manifestations and esophageal function using high-resolution manometry (HRM). METHODS Patients with SSc that had undergone HRM between 1/2004 and 9/2014 were identified and HRMs were analyzed according to the Chicago Classification. Clinical characteristics were identified via retrospective chart review and compared among motility diagnoses while adjusting for age, gender, race, and SSc-disease duration. KEY RESULTS Seventy-nine patients (85% female, ages 25-77) were included. Clinical characteristics were compared between patients with absent contractility (AC, n = 40), ineffective esophageal motility (IEM; n = 15), and normal motility (n = 19); the five remaining patients met criteria for other motility diagnoses. Groups differed in severity of skin involvement measured by the modified Rodnan skin score (0-51): AC (adjusted mean 12.6), IEM (4.4), normal (4.3), p = 0.043. Pulmonary function tests [percent predicted FVC and DLCO) were lower in AC (adjusted mean, FVC: 70.3, DLCO 51.1), than IEM (FVC: 92.0; DLCO: 76.9) and normal motility (FVC: 80.0; DLCO: 67.2), p values 0.057 (FVC) and 0.007 (DLCO). Groups did not differ by SSc-disease duration, autoantibodies, or reported symptoms of dysphagia or reflux. CONCLUSIONS & INFERENCES In patients with SSc, absent esophageal contractility on HRM was associated with increased skin disease severity and worse lung function. Obtaining HRM to identify SSc patients with more severe esophageal dysfunction could be considered to enable implementation of management strategies in patients potentially at risk for increased morbidity and mortality.
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Affiliation(s)
- Jessica N. Kimmel
- Department of Medicine, Division of Gastroenterology and Hepatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Dustin A. Carlson
- Department of Medicine, Division of Gastroenterology and Hepatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Monique Hinchcliff
- Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Mary A. Carns
- Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Kathleen A Aren
- Department of Medicine, Division of Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - Jungwha Lee
- Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
| | - John E. Pandolfino
- Department of Medicine, Division of Gastroenterology and Hepatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA
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18
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Wood RK. Esophageal Dysmotility, Gastro-esophageal Reflux Disease, and Lung Transplantation: What Is the Evidence? Curr Gastroenterol Rep 2016; 17:48. [PMID: 26454656 DOI: 10.1007/s11894-015-0474-9] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Lung transplantation is an effective and life-prolonging therapy for patients with advanced lung disease (ALD). However, long-term patient survival following lung transplantation is primarily limited by development of an inflammatory and fibrotic process involving the lung allograft known as bronchiolitis obliterans syndrome (BOS). Although the precise cause of BOS remains uncertain and is likely multifactorial, chronic aspiration of gastro-duodenal contents is one possible contributing factor. Multiple small, cross-sectional studies performed over the past two decades have reported a high prevalence of gastro-esophageal reflux disease (GERD) and esophageal dysmotility in the ALD population and several investigations suggest the prevalence may increase following lung transplantation. More recent studies evaluating the direct effect of gastro-duodenal contents on airways have demonstrated a possible biologic link between GERD and BOS. Despite the recent advances in our understanding of BOS, further investigations are needed to establish GERD as a causative factor in its development. This review will discuss the existing literature that has identified an association of GERD with ALD and post-transplant populations, with a focus on recent advances in the field.
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Affiliation(s)
- Richard K Wood
- Division of Gastroenterology, Duke University Medical Center, DUMC 3662, Durham, NC, 27710, USA.
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19
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Advances in the evaluation and management of esophageal disease of systemic sclerosis. Curr Rheumatol Rep 2015; 17:475. [PMID: 25475597 DOI: 10.1007/s11926-014-0475-y] [Citation(s) in RCA: 34] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Symptoms of heartburn and dysphagia as well as objective findings of abnormal esophageal acid exposure and esophageal dysmotility are common in patients with systemic sclerosis (SSc). Treatments for SSc esophageal disease are generally limited to gastroesophageal reflux disease (GERD) treatment with proton pump inhibitors. Progresses made in esophageal diagnostic testing offer the potential for improved clinical characterization of esophageal disease in SSc that may help direct management decisions. In addition to reviewing GERD management in patients with SSc, present and potential uses of endoscopy, reflux monitoring, manometry, impedance planimetry, and endoscopic ultrasound are discussed.
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20
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Savarino E, Furnari M, de Bortoli N, Martinucci I, Bodini G, Ghio M, Savarino V. Gastrointestinal involvement in systemic sclerosis. Presse Med 2014; 43:e279-91. [PMID: 25179275 DOI: 10.1016/j.lpm.2014.03.029] [Citation(s) in RCA: 47] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/24/2014] [Accepted: 03/18/2014] [Indexed: 12/12/2022] Open
Abstract
Systemic sclerosis is an autoimmune chronic disease characterised by microvascular, muscular and immunologic abnormalities that lead to progressive and systemic deposition of connective tissue in the skin and internal organs. The gastrointestinal tract is often overlooked by physicians but it is the most affected organ after the skin, from the mouth to the anus. Indeed, 80% of SSc patients may present with gastrointestinal involvement. Gastrointestinal manifestations range from bloating and heartburn to dysphagia and anorectal dysfunction to severe weight loss and malabsorption. However, the gastrointestinal involvement is rarely the direct cause of death, but has great impact on quality of life and leads to several comorbidities that subsequently affect patients' survival. Treatments, including nutritional support and prokinetics provide limited benefits and do not arrest the progressive course of the disease, but earlier detection of gastrointestinal involvement may reduce the risk of complications such as malnutrition.
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Affiliation(s)
- Edoardo Savarino
- Division of Gastroenterology, Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy.
| | - Manuele Furnari
- Division of Gastroenterology, Department of Internal Medicine, University of Genoa, Genoa, Italy
| | - Nicola de Bortoli
- Division of Gastroenterology, Department of Internal Medicine, University of Pisa, Pisa, Italy
| | - Irene Martinucci
- Division of Gastroenterology, Department of Internal Medicine, University of Pisa, Pisa, Italy
| | - Giorgia Bodini
- Division of Gastroenterology, Department of Internal Medicine, University of Genoa, Genoa, Italy
| | - Massimo Ghio
- Division of Internal Medicine, Department of Internal Medicine, University of Genoa, Genoa, Italy
| | - Vincenzo Savarino
- Division of Gastroenterology, Department of Internal Medicine, University of Genoa, Genoa, Italy
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21
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Thomaidis T, Goetz M, Gregor SP, Hoffman A, Kouroumalis E, Moehler M, Galle PR, Schwarting A, Kiesslich R. Irritable bowel syndrome and organic diseases: A comparative analysis of esophageal motility. World J Gastroenterol 2013; 19:6408-6415. [PMID: 24151359 PMCID: PMC3801311 DOI: 10.3748/wjg.v19.i38.6408] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/14/2012] [Revised: 01/24/2013] [Accepted: 05/10/2013] [Indexed: 02/06/2023] Open
Abstract
AIM: To assess the esophageal motility in patients with irritable bowel syndrome (IBS) and to compare those with patients with autoimmune disorders.
METHODS: 15 patients with IBS, 22 with systemic lupus erythematosus (SLE) and 19 with systemic sclerosis (SSc) were prospectively selected from a total of 115 patients at a single university centre and esophageal motility was analysed using standard manometry (Mui Scientific PIP-4-8SS). All patients underwent esophago-gastro-duodenoscopy before entering the study so that only patients with normal endoscopic findings were included in the current study. All patients underwent a complete physical, blood biochemistry and urinary examination. The grade of dysphagia was determined for each patient in accordance to the intensity and frequency of the presented esophageal symptoms. Furthermore, disease activity scores (SLEDAI and modified Rodnan score) were obtained for patients with autoimmune diseases. Outcome parameter: A correlation coefficient was calculated between amplitudes, velocity and duration of the peristaltic waves throughout esophagus and patients’ dysphagia for all three groups.
RESULTS: There was no statistical difference in the standard blood biochemistry and urinary analysis in all three groups. Patients with IBS showed similar pathologic dysphagia scores compared to patients with SLE and SSc. The mean value of dysphagia score was in IBS group 7.3, in SLE group 6.73 and in SSc group 7.56 with a P-value > 0.05. However, the manometric patterns were different. IBS patients showed during esophageal manometry peristaltic amplitudes at the proximal part of esophagus greater than 60 mmHg in 46% of the patients, which was significant higher in comparison to the SLE (11.8%) and SSc-Group (0%, P = 0.003). Furthermore, IBS patients showed lower mean resting pressure of the distal esophagus sphincter (Lower esophageal sphincter, 22 mmHg) when compared with SLE (28 mmHg, P = 0.037) and SSc (26 mmHg, P = 0.052). 23.5% of patients with SLE showed amplitudes greater as 160 mmHg in the distal esophagus (IBS and SSc: 0%) whereas 29.4% amplitudes greater as 100 mmHg in the middle one (IBS: 16.7%, SSc: 5.9% respectively, P = 0.006). Patients with SSc demonstrated, as expected, in almost half of the cases reduced peristalsis or even aperistalsis in the lower two thirds of the esophagus. SSc patients demonstrated a negative correlation coefficient between dysphagia score, amplitude and velocity of peristaltic activity at middle and lower esophagus [r = -0.6, P < 0.05].
CONCLUSION: IBS patients have comparable dysphagia-scores as patients with autoimmune disorders. The different manometric patterns might allow differentiating esophageal symptoms based on IBS from other organic diseases.
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MESH Headings
- Adult
- Autoimmunity
- Deglutition
- Deglutition Disorders/diagnosis
- Deglutition Disorders/etiology
- Deglutition Disorders/physiopathology
- Endoscopy, Digestive System
- Esophageal Sphincter, Lower/physiopathology
- Esophagus/physiopathology
- Female
- Germany
- Humans
- Irritable Bowel Syndrome/complications
- Irritable Bowel Syndrome/diagnosis
- Irritable Bowel Syndrome/physiopathology
- Lupus Erythematosus, Systemic/complications
- Lupus Erythematosus, Systemic/diagnosis
- Lupus Erythematosus, Systemic/immunology
- Lupus Erythematosus, Systemic/physiopathology
- Male
- Manometry
- Middle Aged
- Peristalsis
- Prospective Studies
- Scleroderma, Systemic/complications
- Scleroderma, Systemic/diagnosis
- Scleroderma, Systemic/immunology
- Scleroderma, Systemic/physiopathology
- Severity of Illness Index
- Surveys and Questionnaires
- Time Factors
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22
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Savarino E, Mei F, Parodi A, Ghio M, Furnari M, Gentile A, Berdini M, Di Sario A, Bendia E, Bonazzi P, Scarpellini E, Laterza L, Savarino V, Gasbarrini A. Gastrointestinal motility disorder assessment in systemic sclerosis. Rheumatology (Oxford) 2013; 52:1095-100. [PMID: 23382360 DOI: 10.1093/rheumatology/kes429] [Citation(s) in RCA: 63] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022] Open
Abstract
OBJECTIVES SSc is a clinically heterogeneous and generalized disease, characterized by thickness of the connective tissue of the skin and internal organs, such as the digestive tract, impairing gastrointestinal (GI) motility. Our aim is to evaluate retrospectively abnormalities of oesophageal motility, gastric emptying, oro-cecal transit time (OCTT) and small intestine bacterial overgrowth (SIBO) in a large cohort of SSc patients. METHODS Ninety-nine SSc patients were included in the study. Forty-two patients underwent oesophageal conventional manometry, 45 performed a [(13)C]octanoic acid breath test to measure gastric emptying time and all 99 patients performed a lactulose breath test in order to evaluate OCTT and SIBO. Data were compared with healthy controls. RESULTS In SSc patients, median lower oesophageal sphincter (LOS) pressure [14 mmHg (25th-75th; 8-19) vs 24 mmHg (19-28); P < 0.01] and median wave amplitude [30 mmHg (16-70) vs 72 mmHg (48-96); P < 0.01] were lower than in controls. Oesophageal involvement, defined as reduced LOS pressure and ineffective oesophageal motility pattern, was encountered in 70% of SSc patients. A delayed gastric emptying time was present in 38% of SSc patients: mean t½ was 141 ± 79 min vs 90 ± 40 min of controls (P < 0.01). Also, OCTT was significantly delayed in SSc: median OCTT was 160 min (25th-75th; 135-180) vs 105 min (25th-75th; 90-135) of controls (P < 0.01). SIBO was observed in 46% of SSc compared with 5% of controls (P < 0.01). CONCLUSION GI involvement is very frequent in SSc patients. Oesophagus and small bowel are more frequently impaired, whereas delayed gastric emptying is less common.
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23
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Das L, Bowden A, Cooper RG, Mitchell W, O'Sullivan M, Herrick AL. Percutaneous endoscopic gastrostomy feeding--a life-saving intervention in SSc-myositis overlap with pharyngeal dysfunction. Rheumatology (Oxford) 2012; 51:1518-20. [DOI: 10.1093/rheumatology/kes020] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022] Open
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24
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Anti-cyclic citrullinated peptide antibodies in scleroderma patients. Clin Rheumatol 2012; 31:877-80. [DOI: 10.1007/s10067-011-1930-z] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2011] [Revised: 10/18/2011] [Accepted: 12/22/2011] [Indexed: 02/02/2023]
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25
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Lahcene M, Oumnia N, Matougui N, Boudjella M, Tebaibia A, Touchene B. Esophageal involvement in scleroderma: clinical, endoscopic, and manometric features. ISRN RHEUMATOLOGY 2011; 2011:325826. [PMID: 22389793 PMCID: PMC3263742 DOI: 10.5402/2011/325826] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 05/12/2011] [Accepted: 07/06/2011] [Indexed: 01/01/2023]
Abstract
Aim. To evaluate characteristics of esophageal involvement in scleroderma. Methods. The study was prospective and concerned 194 patients with a definite systemic sclerosis. Gastroesophageal endoscopy and esophageal manometry were performed in all the cases. Results. Symptoms were present in 118 cases (60.8%); they were signs of GERD or dysphagia, respectively, in 94 (48.4%) and 91 patients (46.9%). Reflux esophagitis was found in 73 cases (37.6%); it was mild or moderate in 47 cases (24.2%) and severe or complicated in the remaining cases. Manometry revealed a lower esophageal sphincter incompetence and esophageal motor disorders, respectively, in 118 (60.8%) and 157 cases (80.9%). Presence of these late was not related to age, duration, or skin extension of the disease, but with clinical complaint and/or mucosal damage. Conclusion. Esophageal involvement is frequent during scleroderma. Manometry is the most sensible examination and could be a screening procedure.
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Affiliation(s)
- M Lahcene
- Department of Internal Medicine, Kouba Hospital, Algiers, Algeria
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Thoua NM, Schizas A, Forbes A, Denton CP, Emmanuel AV. Internal anal sphincter atrophy in patients with systemic sclerosis. Rheumatology (Oxford) 2011; 50:1596-602. [PMID: 21504990 DOI: 10.1093/rheumatology/ker153] [Citation(s) in RCA: 43] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023] Open
Abstract
OBJECTIVES SSc is a connective tissue, multisystem disorder of unknown aetiology. The gastrointestinal tract (GIT) is affected in up to 90% of patients. The exact pathophysiology of GIT involvement is not known, but it is related to both neurogenic and myogenic abnormalities as well as possible vascular and ischaemic changes. Thinning of the internal anal sphincter (IAS) has been demonstrated in SSc with faecal incontinence. We aimed to investigate anal sphincter structure in patients with SSc. METHODS Forty-four SSc patients [24 symptomatic (Sx) and 20 asymptomatic (ASx)] and 20 incontinent controls (ICs) were studied. Patients underwent anorectal manometry and endoanal US. RESULTS In the ICs, external anal sphincter defects were more common, but the IAS was less atrophic, evident by both atrophy scores and IAS thickness. There was no significant difference in atrophy scores [Sx: 2 (1.5-3) vs ASx: 2 (1-2)] or IAS thickness [Sx: 1.85 (1.5-2.3) vs ASx: 1.8 (1.7-2.25)] between the Sx and ASx SSc patients. CONCLUSION Patients with SSc (both Sx and ASx) have thin and atrophic IAS, suggesting that IAS atrophy develops even in ASx patients and this may be amenable to treatment with sacral neuromodulation.
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Affiliation(s)
- Nora M Thoua
- GI Physiology Unit, UniversityCollege Hospital, London, UK.
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27
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Cozzi F, Parisi G, Ciprian L, Bullo A, Cardarelli S, Rizzo M, Sfriso P, Punzi L. Gastric dysmotility after liquid bolus ingestion in systemic sclerosis: an ultrasonographic study. Rheumatol Int 2011; 32:1219-23. [PMID: 21258796 DOI: 10.1007/s00296-010-1779-5] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/14/2010] [Accepted: 12/30/2010] [Indexed: 01/15/2023]
Abstract
Gastric involvement appears quite commonly in systemic sclerosis (SSc). The aim of this study was to evaluate gastric wall motility using ultrasonography, a noninvasive method able to track both filling and emptying of fundus and antrum. The study was performed in 20 SSc patients and 20 healthy control subjects. Gastric filling and emptying were evaluated by transabdominal ultrasonography, measuring changes in fundus and antral areas over a 1-h period after ingestion of a liquid bolus (500 ml of mineral water). Areas of both gastric fundus and antrum at basal evaluation were found to be smaller in SSc patients than in healthy controls. Gastric filling was significantly reduced after ingestion of liquid bolus. Gastric emptying was delayed both in fundus and antrum. No significant differences of gastric wall motility have been observed in different subsets of SSc patients. Our findings show that gastric dysmotility is frequent and severe in SSc patients, contributing to the gastrointestinal disturbances which are very common in this disease.
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Affiliation(s)
- Franco Cozzi
- Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Padova, Padova, Italy.
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28
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Castor JM, Wood RK, Muir AJ, Palmer SM, Shimpi RA. Gastroesophageal reflux and altered motility in lung transplant rejection. Neurogastroenterol Motil 2010; 22:841-50. [PMID: 20507544 PMCID: PMC2911519 DOI: 10.1111/j.1365-2982.2010.01522.x] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
BACKGROUND Lung transplantation has become an effective therapeutic option for selected patients with end stage lung disease. Long-term survival is limited by chronic rejection manifest as bronchiolitis obliterans syndrome (BOS). The aspiration of gastric contents has been implicated as a causative or additive factor leading to BOS. Gastroesophageal reflux (GER) and altered foregut motility are common both before and after lung transplantation. Further, the normal defense mechanisms against reflux are impaired in the allograft. Recent studies using biomarkers of aspiration have added to previous association studies to provide a growing body of evidence supporting the link between rejection and GER. Further, the addition of high-resolution manometry (HRM) and impedance technology to characterize bolus transit and the presence and extent of reflux regardless of pH might better identify at-risk patients. Although additional prospective studies are needed, fundoplication appears useful in the prevention or treatment of post-transplant BOS. PURPOSE This review will highlight the existing literature on the relationship of gastroesophageal reflux and altered motility to lung transplant rejection, particularly BOS. The article will conclude with a discussion of the evaluation and management of patients undergoing lung transplantation at our center.
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Affiliation(s)
- John M Castor
- Division of Gastroenterology, Department of Medicine, Duke University Medical Center, Durham, NC, USA
| | - Richard K. Wood
- Division of Gastroenterology, Department of Medicine, Duke University Medical Center, Durham, NC, USA
| | - Andrew J. Muir
- Division of Gastroenterology, Department of Medicine, Duke University Medical Center, Durham, NC, USA
| | - Scott M. Palmer
- Division of Pulmonary and Critical Care, Department of Medicine, Duke University Medical Center, Durham, NC, USA
| | - Rahul A. Shimpi
- Division of Gastroenterology, Department of Medicine, Duke University Medical Center, Durham, NC, USA
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Thoua NM, Bunce C, Brough G, Forbes A, Emmanuel AV, Denton CP. Assessment of gastrointestinal symptoms in patients with systemic sclerosis in a UK tertiary referral centre. Rheumatology (Oxford) 2010; 49:1770-5. [PMID: 20530510 DOI: 10.1093/rheumatology/keq147] [Citation(s) in RCA: 78] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
OBJECTIVES The gastrointestinal tract (GIT) is affected in up to 90% of patients with SSc to a variable extent and severity. We aimed to establish the frequency and range of gastrointestinal (GI) symptoms in SSc patients at the Royal Free Hospital, a tertiary referral centre. METHODS A 52-item, previously validated, questionnaire capturing SSc-related gut dysfunction was given to consecutive patients with SSc attending the rheumatology outpatient department. The questionnaire assesses the 'frequency' of five categories of symptoms and their 'impact' on social functioning and emotional well-being. Patients' notes were reviewed to establish disease subtype, autoantibody profile and other internal organ involvement. RESULTS We collected 402 completed questionnaires (357 females; mean age 55). Sixty-nine per cent of patients had lcSSc and 30% dcSSc with mean disease duration of 11 years. Mean questionnaire scores showed that patients have a wide range of GI symptoms. Ninety-four per cent of patients reported upper and 79% lower GI symptoms, 3% of patients reported no symptoms and 10% reported daily symptoms. There was no association between disease subtype or autoantibody profile and GI symptoms. There was a positive correlation between diarrhoea scores (high scores = best health) and pulmonary fibrosis (r = 0.134, P = 0.0068). No other association between GI symptoms and other internal organ involvement was found. CONCLUSIONS GI symptoms, both upper and lower, are common in patients with SSc. Patients should be asked specifically about GI symptoms as they may be under-reported and therefore under-treated. GI focused questionnaire is an effective way to assess gut symptoms and adjust treatment.
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Affiliation(s)
- Nora M Thoua
- Department of Gastroenterology, University College Hospital, London, UK
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30
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Yin J, Chen JDZ. Gastrointestinal motility disorders and acupuncture. Auton Neurosci 2010; 157:31-7. [PMID: 20363196 DOI: 10.1016/j.autneu.2010.03.007] [Citation(s) in RCA: 142] [Impact Index Per Article: 9.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/11/2009] [Revised: 03/08/2010] [Accepted: 03/09/2010] [Indexed: 12/11/2022]
Abstract
During the last decades, numerous studies have been performed to investigate the effects and mechanisms of acupuncture or electroacupuncture (EA) on gastrointestinal motility and patients with functional gastrointestinal diseases. A PubMed search was performed on this topic and all available studies published in English have been reviewed and evaluated. This review is organized based on the gastrointestinal organ (from the esophagus to the colon), components of gastrointestinal motility and the functional diseases related to specific motility disorders. It was found that the effects of acupuncture or EA on gastrointestinal motility were fairly consistent and the major acupuncture points used in these studies were ST36 and PC6. Gastric motility has been mostly studied, whereas much less information is available on the effect of EA on small and large intestinal motility or related disorders. A number of clinical studies have been published, investigating the therapeutic effects of EA on a number of functional gastrointestinal diseases, such as gastroesophageal reflux, functional dyspepsia and irritable bowel syndrome. However, the findings of these clinical studies were inconclusive. In summary, acupuncture or EA is able to alter gastrointestinal motility functions and improve gastrointestinal motility disorders. However, more studies are needed to establish the therapeutic roles of EA in treating functional gastrointestinal diseases.
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Affiliation(s)
- Jieyun Yin
- Division of Gastroenterology, Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, USA
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31
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Jain R, L. Thiele D. Gastrointestinal and Hepatic Manifestations of Systemic Diseases. SLEISENGER AND FORDTRAN'S GASTROINTESTINAL AND LIVER DISEASE 2010:557-592.e11. [DOI: 10.1016/b978-1-4160-6189-2.00035-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Calderaro DC, de Carvalho MAP, Moretzsohn LD. Esophageal manometry in 28 systemic sclerosis Brazilian patients: findings and correlations. Dis Esophagus 2009; 22:700-4. [PMID: 19664079 DOI: 10.1111/j.1442-2050.2009.01000.x] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Systemic sclerosis (SSc) is a multisystem disease of unknown etiology. Esophageal involvement affects 50-90% of patients and is characterized by abnormal motility and hypotonic lower esophageal sphincter. Data on the association of esophageal abnormalities and age, gender, SSc subset or duration, autoantibody profile, esophageal symptoms, and medication are lacking or conflicting. The aim of this study was the evaluation of these associations in Brazilian sclerodermic patients from the Rheumatology Division, Clinics Hospital, Federal University, Minas Gerais. They underwent medical records review, clinical interview, and esophageal manometry. The normal cutoff level for lower esophageal sphincter pressure was 14 mmHg. Abnormal peristalsis occurred when less than 80% of peristaltic waves were propagated. P-values less than 0.05 were considered significant. Twenty-eight patients were included: 71% were women. The population presented medium age and disease duration of 46 years and 12 years, respectively. Cutaneous diffuse SSc occurred in 39% and its limited form in 61%. Dysphagia, pyrosis, and regurgitation occurred, respectively, in 71%, 43%, and 61% of patients. Lower esophageal sphincter pressure and number of peristaltic waves-propagated medias were, respectively, 17.2 mmHg and 2.3. SSc-related manometric abnormalities were present in 86% of patients. Manometry revealed distal esophageal body hypomotility, hypotonic lower esophageal sphincter, or both, respectively, in 82%, 39%, and 36% of patients. One patient presented the manometric pattern of esophageal achalasia. Male patients more frequently presented hypotonic inferior esophageal sphincter. Manometric findings have had no relationship with the other variables. Nifedipine use did not influence manometric findings.
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Affiliation(s)
- D C Calderaro
- Rheumatology Division of the Department of Musculoskeletal System, Clinics Hospital, School of Medicine, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.
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Esophageal dysmotility in scleroderma: a prospective study of 183 cases. ACTA ACUST UNITED AC 2009; 33:466-9. [PMID: 19540687 DOI: 10.1016/j.gcb.2009.01.014] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2008] [Revised: 12/25/2008] [Accepted: 01/13/2009] [Indexed: 11/23/2022]
Abstract
AIMS The goal of the study was to evaluate the prevalence and risk factors of esophageal motor disorders in systemic sclerosis. PATIENTS AND METHODS In 183 consecutive cases of scleroderma, as diagnosed by American College of Rheumatology criteria (1980). Patients' mean age was 40.6+/-13.3 years, the gender ratio was 0.13 and the average duration of disease was 6.8+/-7.5 years. A localized, cutaneous form was observed in 148 patients (81%) and a diffuse form in 35 (19%). All patients underwent upper gastrointestinal endoscopy and standard esophageal manometry. RESULTS Esophageal symptoms and reflux esophagitis were found in 108 (59%) and 68 (37%) of patients, respectively. Esophageal motor disorders were present in 148 patients (81%), and were associated with a hypotensive lower esophageal sphincter in 114 (62%). The presence of these motor abnormalities was not related to age, gender, skin extension or duration of disease. Esophageal motor disorders were present in almost all patients with esophageal symptoms or esophagitis, and were also found in 48 (64%) of the asymptomatic patients. CONCLUSION Esophageal motor disorders are frequently seen in scleroderma, especially in cases with clinical symptoms, but are not associated with a specific form of the disease.
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Sifrim D, Blondeau K, Mantillla L. Utility of non-endoscopic investigations in the practical management of oesophageal disorders. Best Pract Res Clin Gastroenterol 2009; 23:369-86. [PMID: 19505665 DOI: 10.1016/j.bpg.2009.03.005] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
The current available methods for diagnosis of GORD are symptom questionnaires, catheter and wireless pH-metry, impedance-pH monitoring and Bilitec(@). Osophageal pH monitoring allows both quantitative analysis of acid reflux and assessment of reflux-symptom association. Impedance-pH monitoring detects all types of reflux (acid and non-acid) and allows assessment of proximal extent of reflux, a relevant parameter for understanding symptoms perception and extraoesophageal symptoms. Bilitec provides a quantitative assessment of duodeno-gastro-oesophageal reflux. Oesophageal motor abnormalities have been associated with GORD symptoms as well as chest pain and dysphagia. High-resolution manometry contributed to re-classify oesphageal motor disorders. However, barium swallows are still essential for evaluation of oesophageal anatomy and combined oesophageal manometry-impedance can assess oesophageal motility and bolus transit simultaneously in a non-radiological way. Still in experimental phase, high-frequency ultrasound allows monitoring of the oesophageal wall thickness and exaggerated longitudinal muscle contraction that might be associated to chest pain and dysphagia. This chapter provides a critical evaluation of the clinical application of these techniques.
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Affiliation(s)
- Daniel Sifrim
- Center for Gastroenterological Research, Catholic University of Leuven, Belgium.
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35
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Shitrit D, Amital A, Peled N, Raviv Y, Medalion B, Saute M, Kramer MR. Lung transplantation in patients with scleroderma: case series, review of the literature, and criteria for transplantation. Clin Transplant 2009; 23:178-83. [DOI: 10.1111/j.1399-0012.2009.00958.x] [Citation(s) in RCA: 63] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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Abstract
Esophageal dysphagia can arise from a variety of causes such as motility disorders, mechanical and inflammatory diseases. Adequate management includes a detailed history, evaluation with upper endoscopy, barium radiography and manometry. Treatment is usually tailored to the underlying disease process and in some cases, as in inoperable cancer, palliative management may be necessary.
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Affiliation(s)
- Adeyemi Lawal
- Medical College of Wisconsin, Department of Medicine, Division of Gastroenterology and Hepatology, Froedtert East, FEC-4510, 9200 West Wisconsin Avenue, Milwaukee, WI 53226, USA
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37
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Lidia Vera-Lastra O. [Systemic sclerosis and the gastrointestinal tract. Diagnostic and therapeutic approach]. ACTA ACUST UNITED AC 2008; 2 Suppl 3:S24-30. [PMID: 21794384 DOI: 10.1016/s1699-258x(06)73104-7] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
In systemic sclerosis esophagus is affected in 90% followed by anal and rectal involvement (50-70%), stomach (40-70%), colon (10-50%) and small bowel (40%). The main clinical findings are esophagic: dysphagia and sign of gastroesophageal reflux and its complications. Gastric symptomatology is a consequence of gastroparesis (postprandial fullness, nausea). Intestinal affection produces hypomotility (abdominal distention, deficient intestinal absorption, bacterial overgrowth). In colon there are diverticula, intestinal constipation, and fecal incontinence. For the diagnosis of GIT involvement the following are useful: studies of digestive tract with contrast media, endoscopy of upper GIT; gastric emptying; pH metry; esophageal and rectal manometry, as well as test to investigate deficient intestinal absorption syndrome. The prokinetic are a cornerstone for the treatment of hypomotility of GIT, along with pump proton inhibitors for the esophageal alteration and antibiotics for bacterial overgrowth.
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Affiliation(s)
- Olga Lidia Vera-Lastra
- Departamento de Medicina Interna. Hospital de Especialidades Antonio Fraga Mouret. Centro Médico Nacional La Raza. IMSS. México DF. México
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Abstract
Scleroderma is a generalized autoimmune disease with variable involvement of the skin and major organs (esophagus, lung, heart and kidney). Scleroderma is essentially a skin disease that frequently involves the digestive system. In scleroderma, the esophagus is the most frequently affected organ of the digestive system, and esophageal dysmotility, reflux and stricture may be observed in the advanced stage. Balloon dilatation and bougienage are generally sufficient in patients developing stricture, and the number of cases in whom resection is performed is very low. In a 20-year-old patient with difficulty in taking even liquid foods, tests revealed sclerodermal involvement of the distal end of the esophagus and stricture. Esophageal resection and gastric replacement were performed. Such systemic diseases as scleroderma, although rare, must be considered in the differential diagnosis of nonmalignant dysphagia, and resection must be borne in mind as a surgical option in cases of advanced stricture.
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Affiliation(s)
- Erdal Yekeler
- Department of Thoracic Surgery, Region Training and Research Hospital, Atatürk University, School of Medicine, Erzurum, Turkey
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39
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Gasper WJ, Sweet MP, Golden JA, Hoopes C, Leard LE, Kleinhenz ME, Hays SR, Patti MG. Lung transplantation in patients with connective tissue disorders and esophageal dysmotility. Dis Esophagus 2008; 21:650-5. [PMID: 18459990 DOI: 10.1111/j.1442-2050.2008.00828.x] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Lung and esophageal dysfunction are common in patients with connective tissue disease (CTD). Recent reports have suggested a link between pathologic gastroesophageal reflux and bronchiolitis obliterans syndrome (BOS) after lung transplant. Because patients with CTD have a high incidence of esophageal dysmotility and reflux, this group may be at increased risk of allograft dysfunction after lung transplantation. Little is known about antireflux surgery in these patients. Our aims were to describe: (i) the esophageal motility and reflux profile of patients with CTD referred for lung transplantation; and (ii) the safety and outcomes of laparoscopic fundoplication in this group. A retrospective review of 26 patients with CTD referred for lung transplantation between July 2003 and June 2007 at a single center. Esophageal studies included manometry and ambulatory 24-h pH monitoring. Twenty-three patients had esophageal manometry and ambulatory 24-h pH monitoring. Nineteen patients (83%) had pathologic distal reflux and 7 (30%) also had pathologic proximal reflux. Eighteen patients (78%) had impaired or absent peristalsis. Eleven of 26 patients underwent lung transplantation. Ten patients are alive at a median follow-up of 26 months (range 3-45) and one has bronchiolitis obliterans syndrome-1. Six patients had a laparoscopic fundoplication, 1 before transplantation and 5 after. All fundoplication patients are alive at median follow-up of 25 months (range 19-45). In conclusion, esophageal dysmotility and reflux are common in CTD patients referred for lung transplant. For this group, laparoscopic fundoplication is safe in experienced hands.
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Affiliation(s)
- Warren J Gasper
- Department of Surgery, University of California, San Francisco, CA 94143-0790, USA
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Domsic R, Fasanella K, Bielefeldt K. Gastrointestinal manifestations of systemic sclerosis. Dig Dis Sci 2008; 53:1163-74. [PMID: 17934861 DOI: 10.1007/s10620-007-0018-8] [Citation(s) in RCA: 83] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/02/2007] [Accepted: 09/07/2007] [Indexed: 12/12/2022]
Abstract
Systemic sclerosis is a chronic disorder of connective tissue that affects the gastrointestinal tract in more than 80% of patients. Changes in neuromuscular function with progressive fibrosis of smooth muscle within the muscularis propria impair normal motor function, which may secondarily alter transit and nutrient absorption. Esophageal manifestations with gastroesophageal reflux and dysphagia are the most common visceral manifestation of the disease, often requiring more intense acid-suppressive medication. Gastric involvement may lead to gastroparesis, which can be found in up to 50% of patients. Severe small bowel disease can present as chronic intestinal pseudo-obstruction with distended loops of small intestine, bacterial overgrowth, impaired absorption and progressive development of nutritional deficiencies. While not studied as extensively, systemic sclerosis often also affects colorectal function resulting in constipation, diarrhea or fecal incontinence. Nutritional support and prokinetics have been used with some success to manage gastric and small or large bowel involvement in patients with systemic sclerosis. Despite advances in management, significant gastrointestinal manifestations of systemic sclerosis still carry a poor prognosis with a five-year mortality exceeding 50%.
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Affiliation(s)
- Robyn Domsic
- Division of Rheumatology, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA
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41
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Belloli L, Carlo-Stella N, Ciocia G, Chiti A, Massarotti M, Marasini B. Myocardial involvement in systemic sclerosis. Rheumatology (Oxford) 2008; 47:1070-2. [DOI: 10.1093/rheumatology/ken186] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
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Abstract
Motor abnormalities of the oesophagus are characterised by a chronic impairment of the neuromuscular structures that co-ordinate oesophageal function. The best-defined entity is achalasia, which is discussed in a separate chapter. Other motor disorders with clinical relevance include diffuse oesophageal spasm, oesophageal dysmotility associated with scleroderma, and ineffective oesophageal motility. These non-achalasic motor disorders have variable prevalence but they could be associated with invalidating symptoms such as dysphagia, chest pain and gastro-oesophageal reflux disease. New oesophageal diagnostic techniques, including high-resolution manometry, high-frequency intraluminal ultrasound and intraluminal impedance, allow (1) better definition of peristalsis and sphincter function, (2) assessment of changes in oesophageal wall thickness, and (3) evaluation of pressure gradients within the oesophagus and across the sphincters that can produce normal or abnormal patterns of bolus transport. This chapter discusses recent advances in physiology, pathophysiology, diagnosis and treatment of non-achalasic oesophageal motor disorders.
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Affiliation(s)
- Daniel Sifrim
- Centre for Gastroenterological Research, Catholic University of Leuven, Faculty of Medicine, Belgium.
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Marie I, Ducrotte P, Denis P, Hellot MF, Levesque H. Oesophageal mucosal involvement in patients with systemic sclerosis receiving proton pump inhibitor therapy. Aliment Pharmacol Ther 2006; 24:1593-601. [PMID: 17206947 DOI: 10.1111/j.1365-2036.2006.03180.x] [Citation(s) in RCA: 54] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/01/2023]
Abstract
AIM To assess the prevalence of oesophagitis, Barrett's oesophagus (BE) and other oesophageal mucosal abnormalities in patients with systemic sclerosis (SSc) without prior selection on digestive clinical presentation. We also investigated the association between oesophageal endoscopic and manometric data with clinical manifestations of SSc. METHODS Oesophageal endoscopy and manometry were performed in 133 consecutive patients with SSc, receiving proton pump inhibitor (PPI) therapy since SSc diagnosis. RESULTS Endoscopy revealed oesophagitis in 43 patients (32.3%), BE in 9 patients (6.8%), candidiasis in 7 patients (5.3%) and hyperplastic polyp arising in ectopic gastric mucosa in 1 patient. Patients with severe oesophageal motor impairment further exhibited a higher prevalence of interstitial lung disease (ILD) when compared with those without. CONCLUSION Our study underlines the high frequency of oesophageal mucosal abnormalities in unselected SSc patients receiving long-term PPI therapy. A relationship between oesophagitis/BE and severe manometric motor disturbances was established; these patients may require a higher regimen of PPI. Finally, our series indicates a correlation between severe oesophageal motor disturbances and evidence for ILD in patients, suggesting that gastro-oesophageal reflux may be one of the contributing factors of ILD in SSc; this subgroup of patients may require close monitoring of lung parameters.
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Affiliation(s)
- I Marie
- Department of Internal Medicine, Rouen University Hospital, Rouen Cedex, France.
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Abstract
Progressive systemic sclerosis (PSS) causes smooth muscle atrophy and fibrosis of the distal two-thirds of the esophagus. Motility studies show reduced-amplitude or absent peristaltic contractions in this region and normal or decreased lower esophageal sphincter pressure. Patients complain of dysphagia, heartburn, and regurgitation due to reflux and dysmotility. Complications include strictures found in 17% to 29% of patients and Barrett esophagus is 0% to 37%. Candida esophagitis is a complication of PSS not seen with non-PSS reflux. Esophageal disease correlates with pulmonary involvement but not with disease in the stomach or intestines. Whether reflux contributes to the pulmonary disease is an open question. Although manometry is the gold standard for diagnosis, cine-esophagram and scintography are only slightly less sensitive and should be considered for following the patients. Symptoms correlate poorly with evidence of esophagitis or abnormal 24-hour pH recordings. As a result, it is unclear which patients should receive acid-reducing or prokinetic medications and which medication to use. Aspiration precautions are important in those with severe esophageal dysmotility. This review of the literature highlights many areas of uncertainty in the diagnosis and treatment of esophageal disease in PSS that can be addressed in clinical studies.
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Affiliation(s)
- Ellen C Ebert
- UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08903, USA.
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45
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Bassotti G, D'Onofrio V, Battaglia E, Fiorella S, Dughera L, Iaquinto G, Mazzocchi A, Morelli A, Annese V. Treatment with botulinum toxin of octo-nonagerians with oesophageal achalasia: a two-year follow-up study. Aliment Pharmacol Ther 2006; 23:1615-1619. [PMID: 16696811 DOI: 10.1111/j.1365-2036.2006.02907.x] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
BACKGROUND Treatment of oesophageal achalasia with intrasphincteric injections of botulinum toxin has proved to be a successful alternative treatment modality. However, little is known about its long-term effects in very old patients. AIM To evaluate the effects of such treatment in octo-nonagerians during a 2-year follow-up period. PATIENTS AND METHODS Thirty-three patients with idiopathic oesophageal achalasia (range 81-94 years) entered the study. After basal evaluation and screening procedures, 100 U of botulinum toxin was injected at the lower oesophageal sphincter, and the procedure was repeated 1 month later. Data were collected at baseline and were compared after 1 and 2 years following the procedure. RESULTS Seventy-eight per cent of patients were considered responders at 1 year and 54% were considered responders at 2 years. The weight gain at the end of the follow-up period was 2 (0-3) kg. No significant relationship was found between baseline lower oesophageal sphincter pressure and symptoms score after 1 and 2 years of follow-up; moreover, no major complications of botulinum toxin therapy were reported. CONCLUSION Treatment of very old achalasic patients with botulinum toxin is safe, effective and yields good quality of life in a substantial proportion of these subjects.
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Affiliation(s)
- G Bassotti
- Gastroenterology and Hepatology Section, Department of Clinical and Experimental Medicine, University of Perugia, Perugia, Italy.
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Sallam H, McNearney TA, Chen JDZ. Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma). Aliment Pharmacol Ther 2006; 23:691-712. [PMID: 16556171 DOI: 10.1111/j.1365-2036.2006.02804.x] [Citation(s) in RCA: 128] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Gastrointestinal dysmotility in systemic sclerosis (scleroderma) is prevalent in 90% of patients, increasing morbidity and in some cases mortality. The resultant gastrointestinal complications are usually extensive, involving many regions of the gut from the oesophagus to the anus. Collagen replacement of vascular and enteric smooth muscle results in hypomotility, lumen dilatation, tensile rigidity and eventual loss of organ functions. The aim of this paper is to provide an overview of systemic sclerosis-related gastrointestinal dysmotility and available/potential therapeutic options. We evaluated published data on the pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis patients using the MEDLINE database for English and non-English articles from 1966 to July 2005. Based on this systematic review, lifestyle and medical therapy approaches are preferred as they often improve and/or ameliorate symptoms. Surgery is only recommended with serious, rare complications such as bowel perforation or ischaemia. Alternative therapies such as acupuncture-based therapies are well tolerated, with clinical improvement and may be of potential therapeutic benefit for systemic sclerosis gastrointestinal dysmotility. Further elucidation of initiating and persistent mechanisms of systemic sclerosis-related gastrointestinal dysmotility will optimize the development of a multidisciplinary and more directed treatment regimen.
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Affiliation(s)
- H Sallam
- Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX 77555-0632, USA
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Wipff J, Allanore Y, Soussi F, Terris B, Abitbol V, Raymond J, Chaussade S, Kahan A. Prevalence of Barrett's esophagus in systemic sclerosis. ACTA ACUST UNITED AC 2005; 52:2882-8. [PMID: 16142744 DOI: 10.1002/art.21261] [Citation(s) in RCA: 55] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
OBJECTIVE The esophagus is the most commonly affected gastrointestinal area in systemic sclerosis (SSc). Patients with SSc frequently develop gastroesophageal reflux, esophageal injury, and sometimes, intestinal metaplasia, or Barrett's esophagus (BE), which may increase the risk of esophageal adenocarcinoma. This study sought to determine the prevalence of BE and esophageal adenocarcinoma in a cohort of SSc patients. METHODS One hundred ten SSc patients who were receiving long-term treatment with proton-pump inhibitors (PPIs) were assessed systematically by esophageal manometry and endoscopy. Esophageal biopsies were performed when macroscopic abnormalities were detected, and BE was diagnosed histologically. RESULTS Among the 110 patients, 14 had BE (12.7%). None of the patients with BE had adenocarcinoma, but 3 of the 14 patients (21%) had dysplasia on esophageal biopsy. Similar proportions of patients with and without BE had abnormal peristalsis and decreased lower esophageal sphincter pressure. No association between BE and other disease characteristics was demonstrated. CONCLUSION In this study, 12.7% of SSc patients who had been treated with PPIs for long periods had BE, similar to the prevalence in patients with gastroesophageal reflux disease. Although none of the patients had esophageal adenocarcinoma, patients with BE should be followed up closely, particularly patients with dysplasic BE. Long-term prospective studies are warranted to determine the phenotype of SSc patients at high risk of developing dysplasia or esophageal adenocarcinoma.
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Affiliation(s)
- J Wipff
- Cochin Hospital, AP-HP Paris 5 University, Paris, France
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Sjölund K, Bartosik I, Lindberg G, Scheja A, Wildt M, Akesson A. Small intestinal manometry in patients with systemic sclerosis. Eur J Gastroenterol Hepatol 2005; 17:1205-12. [PMID: 16215433 DOI: 10.1097/00042737-200511000-00009] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
OBJECTIVES The study explores, by the use of manometry, the frequency and severity of small intestinal involvement in patients with systemic sclerosis, and relates the manometric findings to clinical symptoms, radiology, and some intestinal regulatory peptides. METHODS Stationary antroduodeno-jejunal manometry was used to study small bowel involvement in 10 patients with systemic sclerosis and dysmotility of the oesophagus or signs of malabsorption. Measurements were made during fasting, after a meal, and after octreotide administration and were then compared with a sex-matched control group of healthy individuals. Plasma samples were taken in order to analyse levels of motilin, peptide YY, cholecystokinin, and somatostatin. RESULTS Manometry was abnormal, with signs of intestinal pseudo-obstruction in eight out of 10 patients. In the control group, one individual had an abnormal manometry, as a result of burst activity. The mean contractile amplitudes during fasting and periods after food, spontaneous phase III periods, and octreotide-induced activity complexes were significantly reduced in the systemic sclerosis group when compared with controls. None of the patients, including two with advanced manometric intestinal disturbances, had small intestinal dilatation when examined by radiography. The plasma peptide levels did not differ significantly between the two groups. CONCLUSIONS In eight out of 10 patients the manometric criteria for intestinal pseudo-obstruction were fulfilled, with a motility pattern consistent with both neuropathy and myopathy. The release of motility-regulating peptides was unaffected.
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Affiliation(s)
- Kristina Sjölund
- Department of Internal Medicine, Lund University Hospital, Sweden.
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Dughera L, Battaglia E, Maggio D, Cassolino P, Mioli PR, Morelli A, Emanuelli G, Bassotti G. Botulinum toxin treatment of oesophageal achalasia in the old old and oldest old: a 1-year follow-up study. Drugs Aging 2005; 22:779-783. [PMID: 16156681 DOI: 10.2165/00002512-200522090-00006] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
BACKGROUND Intrasphincteric injection of botulinum toxin (BTX) has become one of the most frequent therapeutic approaches for the treatment of oesophageal achalasia. This treatment seems particularly effective in elderly patients who are not candidates for more invasive procedures. AIMS There are few or no data on BTX treatment of achalasia in the old old and oldest old. Therefore, we evaluated BTX treatment in a group of patients with achalasia in the extreme age range who were too ill or frail to undergo surgery or pneumatic dilatation. PATIENTS AND METHODS Twelve elderly achalasic patients (age range 81-94 years, average age 86 years) with American Society of Anesthesiologists (ASA) class III-IV status were recruited for the study. After baseline clinical and instrumental evaluations, BTX 100U was injected at time 0 and 1 month later. Clinical follow-up was carried out after 3, 6 and 12 months. RESULTS A significant improvement in symptom score was documented at each follow-up step. On the basis of improvements in scores, approximately 70% of patients were considered responders at the end of follow-up. CONCLUSIONS BTX treatment is an effective treatment in a substantial proportion of achalasic patients >80 years of age, in whom benefits are still detectable after 12 months. BTX is a therapeutic option in patients unsuitable for surgery or pneumatic dilatation.
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Affiliation(s)
- Luca Dughera
- Department of Gastroenterology and Clinical Nutrition, University of Torino, Torino, Italy
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Airò P, Della Casa D, Danieli E, Missale G, Cattaneo R, Cestari R. Oesophageal manometry in early and definite systemic sclerosis. Clin Rheumatol 2004; 24:370-6. [PMID: 15592901 DOI: 10.1007/s10067-004-1049-6] [Citation(s) in RCA: 18] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2004] [Accepted: 10/12/2004] [Indexed: 10/26/2022]
Abstract
The objective of this study was to evaluate the oesophageal dysfunction in patients with "early" systemic sclerosis (SSc), as defined by LeRoy and Medsger, to compare it with that of patients with definite SSc, and to correlate it with other features of the disease. Oesophageal manometry results were retrospectively evaluated in 181 patients classified by the 2001 LeRoy and Medsger criteria and the 1980 American College of Rheumatology (ACR) criteria: group 1: limited SSc: Raynaud's phenomenon plus specific nailfold capillaroscopy abnormalities and/or autoantibodies; group 2: limited cutaneous SSc not satisfying the ACR criteria (lcSSc ACR-); group 3: lcSSc ACR+; group 4: diffuse cutaneous SSc. Peristaltic abnormalities in the oesophageal body were present in 73 of 125 patients with SSc ACR+ (groups 3 and 4) compared with 13 of 56 with SSc ACR- (groups 1 and 2) (p < 0.0001). They were more severe in patients with more advanced disease (1 vs 2; 1 vs 3; 1 vs 4; 2 vs 4; p < 0.05) and in patients anti-Scl-70+ than in patients anticentromere positive (p = 0.02). Abnormalities of the lower oesophageal sphincter (LES) were present in 35 of 125 patients with SSc ACR+ and 11 of 56 with SSc ACR- (not statistically different). They were correlated with forced vital capacity (FVC) (LES pressure: p = 0.0005; LES length: p = 0.0004). Abnormalities of the oesophageal body and of the LES were found in 21 and 16% of 46 patients without oesophageal symptoms. Oesophageal manometry can detect abnormalities in a sizeable proportion of patients with "early SSc" not fulfilling the ACR criteria, including asymptomatic patients. The correlation between LES abnormalities and FVC suggests a possible causal relationship between these disease manifestations.
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Affiliation(s)
- Paolo Airò
- The Rheumatology, Allergology and Clinical Immunology Service, Spedali Civili and University, Brescia, Italy.
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