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Good SD, Lee JY, Johnson RE, Volkmann ER. A scoping review of the epidemiology of systemic sclerosis and its organ manifestations: 2018-2024. Curr Opin Rheumatol 2025; 37:103-112. [PMID: 39470126 PMCID: PMC11779589 DOI: 10.1097/bor.0000000000001063] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/30/2024]
Abstract
PURPOSE OF REVIEW Updates from large, observational cohorts and new statistical techniques have resulted in new data on the epidemiology of systemic sclerosis (SSc). This scoping review uses data from 2018 to 2024 to describe the current understanding of the epidemiology of SSc and several of its organ- manifestations. RECENT FINDINGS Our review identified new estimates for the global incidence and prevalence of SSc (1.4-8.6 per 100 000 person-years and 17.6-18.9 per 100 000 individuals, respectively). Mortality rates remain high, though mortality at younger ages has decreased. interstitial lung disease and pulmonary arterial hypertension remain the most common causes of death for patients with SSc. Literature on gastrointestinal (GI) manifestations of SSc was scarce, and we identified significant heterogeneity in results. Furthermore, data on the epidemiology of racial, ethnic and sex-based disparities was lacking. SUMMARY New techniques for the evaluation of the epidemiology of SSc highlight the high morbidity and mortality of SSc, and a growing prevalence rate compared with prior eras. Further research is needed to address notable heterogeneity in the reporting of epidemiological data and understudied disease manifestations, including GI disease and health disparities in disease outcomes.
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Affiliation(s)
- Samuel D Good
- Division of Rheumatology, Department of Medicine, David Geffen School of Medicine
| | | | - Robert E Johnson
- Louise M. Darling Biomedical Library, University of California Los Angeles, Los Angeles, California, USA
| | - Elizabeth R Volkmann
- Division of Rheumatology, Department of Medicine, David Geffen School of Medicine
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2
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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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Uchiyama K, Ando T, Kishimoto E, Nishimura T, Imamoto E, Takagi T, Ishikawa T, Naito Y, Itoh Y. Correlation of gastrointestinal symptom rating scale and frequency scale for the symptoms of gastroesophageal reflux disease with endoscopic findings. Scand J Gastroenterol 2024; 59:1220-1228. [PMID: 39301940 DOI: 10.1080/00365521.2024.2406537] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/06/2024] [Revised: 09/10/2024] [Accepted: 09/15/2024] [Indexed: 09/22/2024]
Abstract
BACKGROUND Gastroesophageal Reflux Disease (GERD) is caused by the reflux of gastric contents into the esophagus and has a 13% global prevalence that is increasing. GERD symptoms negatively impact physical, social, and emotional quality of life. The Frequency Scale for the Symptoms of GERD (FSSG) and the Gastrointestinal Symptom Rating Scale (GSRS) determine the efficacy of treatment but may not correlate with endoscopically estimated esophageal mucosal injury severity. We aimed to probe the correlation between FSSG, GSRS, and esophageal injury severity to evaluate whether these scores can predict GERD severity. METHODS A total of 2962 patients who underwent physical examinations, including upper gastrointestinal endoscopy, at the Kyoto Kuramaguchi Medical Center, Japan, were enrolled in this study. Upper gastrointestinal endoscopy was used to diagnose fundic mucosal atrophy, reflux esophagitis based on the Los Angeles (LA) classification, gastroesophageal flap value function (GEFV) based on Hill's classification, and Barrett's esophagus. Endoscopic diagnoses were examined for correlations with FSSG and GSRS scores. RESULTS In reflux esophagitis, FSSG and GSRS scores correlated with LA-B and LA-C endoscopic diagnosis but not with LA-M and LA-A endoscopic findings. Multiple regression analysis results were similar. FSSG scores reflected advanced fundic gland mucosal atrophy, while GSRS scores associated with high grade of GEFV. CONCLUSIONS This is the first report to examine the correlation between FSSG and GSRS scores and endoscopic findings in a relatively large patient population. Our findings suggest that these scores can diagnose the severity of reflux esophagitis.
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Affiliation(s)
- Kazuhiko Uchiyama
- Molecular Gastroenterology and Hepatology, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | | | - Etsuko Kishimoto
- Center for Health Promotion, Japanses Red Cross Kyoto Daiichi Hospital, Kyoto, Japan
| | | | | | - Tomohisa Takagi
- Molecular Gastroenterology and Hepatology, Kyoto Prefectural University of Medicine, Kyoto, Japan
- Department for Medical Innovation and Translational Medical Science, Graduate School of Medical Science, Kyoto, Japan
| | - Takeshi Ishikawa
- Molecular Gastroenterology and Hepatology, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Yuji Naito
- Department of Human Immunology and Nutrition Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Yoshito Itoh
- Molecular Gastroenterology and Hepatology, Kyoto Prefectural University of Medicine, Kyoto, Japan
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Witarto AP, Witarto BS, Pramudito SL, Ratri LC, Wairooy NAP, Konstantin T, Putra AJE, Wungu CDK, Mufida AZ, Gusnanto A. Risk factors and 26-years worldwide prevalence of endoscopic erosive esophagitis from 1997 to 2022: a meta-analysis. Sci Rep 2023; 13:15249. [PMID: 37709957 PMCID: PMC10502104 DOI: 10.1038/s41598-023-42636-7] [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: 06/23/2023] [Accepted: 09/13/2023] [Indexed: 09/16/2023] Open
Abstract
Erosive esophagitis (EE) is the part of gastroesophageal reflux disease (GERD) spectrum and may progress to esophageal adenocarcinoma. Due to its progressivity and unclear prevalence, we aim to identify the factors contributing in EE to decide the need for further examination. We performed a PRISMA 2020-based systematic search through PubMed and other resources up to June 2, 2022. Study quality was assessed using the Newcastle-Ottawa Scale (NOS). The odds ratio (OR) of each factor and worldwide prevalence of EE were measured. There are 114 observational studies included with a total of 759,100 participants. Out of 29 factors, the significant risk factors are age ≥ 60 y.o. (OR 2.03 [1.81-2.28]), White/Caucasian (OR 1.67 [1.40-1.99]), unmarried (OR 1.08 [1.03-1.14]), having GERD ≥ 5 years (OR 1.27 [1.14-1.42]), general obesity (OR 1.78 [1.61-1.98]), central obesity (OR 1.29 [1.18-1.42]), diabetes mellitus (DM) (OR 1.24 [1.17-1.32]), hypertension (OR 1.16 [1.09-1.23]), dyslipidemia (OR 1.15 [1.06-1.24]), hypertriglyceridemia (OR 1.42 [1.29-1.57]), hiatal hernia (HH) (OR 4.07 [3.21-5.17]), and non-alcoholic fatty liver disease (NAFLD) (OR 1.26 [1.18-1.34]). However, H. pylori infection (OR 0.56 [0.48-0.66]) and atrophic gastritis (OR 0.51 [0.31-0.86]) are protective towards EE. This study demonstrates that age, ethnicity, unmarried, long-term GERD, metabolic diseases, HH, and NAFLD act as risk factors for EE, whereas H. pylori infection and atrophic gastritis act as protective factors. These findings may enable a better understanding of EE and increase greater awareness to address its growing burden.
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Affiliation(s)
| | | | | | | | | | - Tiffany Konstantin
- Medical Program, Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia
| | | | - Citrawati Dyah Kencono Wungu
- Department of Physiology and Medical Biochemistry, Universitas Airlangga, Jl. Mayjen Prof. Dr. Moestopo No. 47, Surabaya, 60132, Indonesia.
- Institute of Tropical Disease, Universitas Airlangga, Surabaya, Indonesia.
| | - Annisa Zahra Mufida
- Department of Internal Medicine, Dr. Soetomo General Hospital, Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia
- Department of Internal Medicine, Universitas Airlangga Hospital, Faculty of Medicine, Universitas Airlangga, Surabaya, Indonesia
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Muacevic A, Adler JR, Figueira ER. First Robotic Roux-en-Y Gastric Bypass for the Treatment of Refractory Gastroesophageal Reflux Disease in a Patient With Systemic Sclerosis. Cureus 2023; 15:e33861. [PMID: 36819430 PMCID: PMC9933002 DOI: 10.7759/cureus.33861] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/17/2023] [Indexed: 01/19/2023] Open
Abstract
Systemic sclerosis (SSc) is an immune-mediated disease that results in fibrosis of the skin and internal organs. Refractory gastroesophageal reflux disease (GERD) associated with severe esophageal dysmotility is common in SSc patients, and surgical treatment with usual anti-reflux procedures such as fundoplications is associated with dismal symptomatic relief and postoperative dysphagia. We report the first robotic short-limb Roux-en-Y gastric bypass (RYGB) with a short Roux limb for the treatment of GERD in a patient with SSc with intense esophageal dysmotility. The operative time was two hours. The procedure and postoperative course were uneventful. The patient presented complete relief of gastroesophageal reflux symptoms and no postoperative dysphagia in a two-year follow-up. Therefore, short-limb RYGB is a safe and very effective alternative for the treatment of severe GERD in patients with SSc. The robotic surgical platform may have some advantages compared to conventional laparoscopy.
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Rankouhi TR, Keulen DV, Tempel D, Venhorst J. Oncostatin M: Risks and Benefits of a Novel Therapeutic Target for Atherosclerosis. Curr Drug Targets 2022; 23:1345-1369. [PMID: 35959619 DOI: 10.2174/1389450123666220811101032] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2022] [Revised: 05/30/2022] [Accepted: 06/03/2022] [Indexed: 01/25/2023]
Abstract
BACKGROUND Cardiovascular disease (CVD) is a leading cause of death worldwide. It is predicted that approximately 23.6 million people will die from CVDs annually by 2030. Therefore, there is a great need for an effective therapeutic approach to combat this disease. The European Cardiovascular Target Discovery (CarTarDis) consortium identified Oncostatin M (OSM) as a potential therapeutic target for atherosclerosis. The benefits of modulating OSM - an interleukin (IL)-6 family cytokine - have since been studied for multiple indications. However, as decades of high attrition rates have stressed, the success of a drug target is determined by the fine balance between benefits and the risk of adverse events. Safety issues should therefore not be overlooked. OBJECTIVE In this review, a risk/benefit analysis is performed on OSM inhibition in the context of atherosclerosis treatment. First, OSM signaling characteristics and its role in atherosclerosis are described. Next, an overview of in vitro, in vivo, and clinical findings relating to both the benefits and risks of modulating OSM in major organ systems is provided. Based on OSM's biological function and expression profile as well as drug intervention studies, safety concerns of inhibiting this target have been identified, assessed, and ranked for the target population. CONCLUSION While OSM may be of therapeutic value in atherosclerosis, drug development should also focus on de-risking the herein identified major safety concerns: tissue remodeling, angiogenesis, bleeding, anemia, and NMDA- and glutamate-induced neurotoxicity. Close monitoring and/or exclusion of patients with various comorbidities may be required for optimal therapeutic benefit.
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Affiliation(s)
- Tanja Rouhani Rankouhi
- Department of Risk Analysis for Products in Development, TNO, Utrechtseweg 48, 3704 HE, Zeist, The Netherlands
| | - Daniëlle van Keulen
- SkylineDx BV, Science and Clinical Development, 3062 ME Rotterdam, The Netherlands
| | - Dennie Tempel
- SkylineDx BV, Science and Clinical Development, 3062 ME Rotterdam, The Netherlands
| | - Jennifer Venhorst
- Department of Risk Analysis for Products in Development, TNO, Utrechtseweg 48, 3704 HE, Zeist, The Netherlands
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7
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Bernal-Bello D, Serantes-Gómez D, Izquierdo-Martínez A, Morales-Ortega A, Duarte-Millán MÁ, Frutos-Pérez B. Letter to the Editor Regarding Esophageal Dysfunction and Systemic Sclerosis: Drugs Should be Kept in Mind. Rheumatol Ther 2022; 9:1237-1240. [PMID: 35716236 PMCID: PMC9314470 DOI: 10.1007/s40744-022-00459-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/15/2021] [Accepted: 04/26/2022] [Indexed: 02/07/2023] Open
Affiliation(s)
- David Bernal-Bello
- Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Camino del Molino, 2. 28942, Fuenlabrada, Madrid, Spain.
| | - David Serantes-Gómez
- Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Camino del Molino, 2. 28942, Fuenlabrada, Madrid, Spain
| | - Aida Izquierdo-Martínez
- Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Camino del Molino, 2. 28942, Fuenlabrada, Madrid, Spain
| | - Alejandro Morales-Ortega
- Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Camino del Molino, 2. 28942, Fuenlabrada, Madrid, Spain.,Department of Medicine, Universidad de Alcalá, Madrid, Spain
| | - Miguel Ángel Duarte-Millán
- Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Camino del Molino, 2. 28942, Fuenlabrada, Madrid, Spain
| | - Begoña Frutos-Pérez
- Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Camino del Molino, 2. 28942, Fuenlabrada, Madrid, Spain
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8
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Yamamichi N, Shimamoto T, Takahashi Y, Takahashi M, Takeuchi C, Wada R, Fujishiro M. Trends in proton pump inhibitor use, reflux esophagitis, and various upper gastrointestinal symptoms from 2010 to 2019 in Japan. PLoS One 2022; 17:e0270252. [PMID: 35714110 PMCID: PMC9205527 DOI: 10.1371/journal.pone.0270252] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2022] [Accepted: 06/07/2022] [Indexed: 11/18/2022] Open
Abstract
The increasing usage of proton pump inhibitors (PPIs) has been reported worldwide, but information on PPI use in East Asia is inadequate. This study aimed to examine the trends in PPI use in Japan, along with the changes in histamine H2 receptor antagonist (H2RA) use, disease rate of reflux esophagitis, and the prevalence of upper gastrointestinal symptoms. We analyzed 217,712 healthy subjects (127,607 men and 90,105 women; 51.4 ± 9.7 years old) participating in the health check program from 2010 to 2019. Various upper gastrointestinal symptoms were evaluated using the frequency scale for the symptoms of gastroesophageal reflux disease (FSSG) questionnaire. Reflux esophagitis was diagnosed by esophageal erosion using the Los Angeles classification grades A, B, C, and D. From 2010 to 2019, the percentage of PPI users increased markedly from approximately 1.8% to 5.3%, whereas that of H2RA users decreased gradually from approximately 2.5% to 1.9%. The use of all classical types of PPIs (omeprazole, lansoprazole, rabeprazole, and esomerazole) and a new type of PPI, a potassium-competitive acid blocker (vonoprazan), greatly increased during the 10 years. An upward trend in the prevalence of reflux esophagitis was observed from 2010 to 2015, but not from 2016 to 2019, indicating that the monotonic rising prevalence of reflux disease stopped in the middle of the 2010s in Japan. In contrast, various upper gastrointestinal symptoms significantly improved between 2010 and 2019. All 12 FSSG symptoms of PPI users were significantly worse than those of non-PPI users, suggesting that PPIs still cannot completely control upper gastrointestinal symptoms. In conclusion, this study revealed a significant increase in PPI use and a slight decrease in H2RA use from 2010 to 2019. Despite a plateau in the prevalence of reflux esophagitis and considerable improvement in various upper gastrointestinal symptoms, PPI use has continued to increase in Japan.
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Affiliation(s)
- Nobutake Yamamichi
- Center for Epidemiology and Preventive Medicine, The University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
- * E-mail:
| | - Takeshi Shimamoto
- Center for Epidemiology and Preventive Medicine, The University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan
- Kameda Medical Center Makuhari CD-2, Mihama-ku, Chiba-City, Japan
| | - Yu Takahashi
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Mami Takahashi
- Center for Epidemiology and Preventive Medicine, The University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan
| | - Chihiro Takeuchi
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
| | - Ryoichi Wada
- Kameda Medical Center Makuhari CD-2, Mihama-ku, Chiba-City, Japan
| | - Mitsuhiro Fujishiro
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, Japan
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Kadakuntla A, Juneja A, Sattler S, Agarwal A, Panse D, Zakhary N, Pasumarthi A, Shapiro L, Tadros M. Dysphagia, reflux and related sequelae due to altered physiology in scleroderma. World J Gastroenterol 2021; 27:5201-5218. [PMID: 34497445 PMCID: PMC8384755 DOI: 10.3748/wjg.v27.i31.5201] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2021] [Revised: 05/13/2021] [Accepted: 07/30/2021] [Indexed: 02/06/2023] Open
Abstract
Systemic sclerosis is a connective tissue disease that presents with significant gastrointestinal involvement, commonly in the esophagus. Dysphagia is a common clinical manifestation of systemic sclerosis and is strongly related to esophageal dysmotility. However, there are multiple other contributing factors in each step in the physiology of swallowing that may contribute to development of severe dysphagia. The oral phase of swallowing may be disrupted by poor mastication due to microstomia and poor dentition, as well as by xerostomia. In the pharyngeal phase of swallowing, pharyngeal muscle weakness due to concurrent myositis or cricopharyngeal muscle tightening due to acid reflux can cause disturbance. The esophageal phase of swallowing is most commonly disturbed by decreased peristalsis and esophageal dysmotility. However, it can also be affected by obstruction from chronic reflux changes, pill-induced esophagitis, or Candida esophagitis. Other contributing factors to dysphagia include difficulties in food preparation and gastroparesis. Understanding the anatomy and physiology of swallowing and evaluating systemic sclerosis patients presenting with dysphagia for disturbances in each step can allow for development of better treatment plans to improve dysphagia and overall quality of life.
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Affiliation(s)
| | - Ankit Juneja
- Albany Medical College, Albany, NY 12208, United States
| | | | | | - Drishti Panse
- Albany Medical College, Albany, NY 12208, United States
| | - Nardin Zakhary
- Department of Dentistry, Ministry of Health, Alexandria 21500, Egypt
| | | | - Lee Shapiro
- Division of Rheumatology, Albany Medical Center, Albany, NY 12208, United States
| | - Micheal Tadros
- Division of Gastroenterology, Albany Medical Center, Albany, NY 12208, United States
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10
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Voulgaris TA, Karamanolis GP. Esophageal manifestation in patients with scleroderma. World J Clin Cases 2021; 9:5408-5419. [PMID: 34307594 PMCID: PMC8281422 DOI: 10.12998/wjcc.v9.i20.5408] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2021] [Revised: 04/22/2021] [Accepted: 05/10/2021] [Indexed: 02/06/2023] Open
Abstract
The esophagus is the most commonly affected part of the gastrointestinal system in patients with systemic sclerosis (SSc). Esophageal involvement may lead to a significant reduction in patient quality of life. The exact pathophysiology is complex and not yet fully elucidated. Ultimately, esophageal smooth muscle becomes atrophied and replaced by fibrous tissue leading to severe motility disturbance of the distal esophagus. Symptoms are mainly attributed to gastroesophageal reflux disease and to esophageal dysmotility. Compelling evidence has correlated esophageal involvement to the severity of pulmonary disease. No formed guidelines exist about the diagnostic modalities used to assess esophageal disease in patients with SSc, though upper gastrointestinal endoscopy is the first and most important modality used as it can reveal alterations commonly observed in patients with SSc. Further exploration can be made by high resolution manometry and pH-impedance study. Proton pump inhibitors remain the mainstay of treatment, while prokinetic agents are commonly used as add-on therapy in patients with symptoms attributed to gastroesophageal reflux disease not responding to standard therapy as well as to motility disturbances. Gastroesophageal reflux disease symptoms in patients with SSc are frequently difficult to manage, and new therapeutic modalities are emerging. The role of surgical treatment is restricted and should only be preserved for resistant cases.
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Affiliation(s)
- Theodoros A Voulgaris
- Department of Gastroenterology and Hepatology, Laiko General Hospital, National and Kapodistian University of Athens, Athens 11527, Greece
| | - Georgios P Karamanolis
- Department of Gastroenterology and Hepatology, Laiko General Hospital, National and Kapodistian University of Athens, Athens 11527, Greece
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11
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Akiyama J, Sumida J, Nakagawa K, Masamune A, Issariyakulkarn N, Patcharatrakul T, Shetler K, Kuribayashi S, Uraoka T, Triadafilopoulos G. New developments in esophageal function testing and esophageal manifestations of connective tissue disorders. Ann N Y Acad Sci 2020; 1481:170-181. [DOI: 10.1111/nyas.14424] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2020] [Revised: 05/25/2020] [Accepted: 06/09/2020] [Indexed: 12/31/2022]
Affiliation(s)
- Junichi Akiyama
- Division of Gastroenterology and Hepatology National Center for Global Health and Medicine Tokyo Japan
| | - Junko Sumida
- Division of Gastroenterology and Hepatology National Center for Global Health and Medicine Tokyo Japan
| | - Kenichiro Nakagawa
- Division of Gastroenterology Tohoku University Graduate School of Medicine Sendai Japan
| | - Atsushi Masamune
- Division of Gastroenterology Tohoku University Graduate School of Medicine Sendai Japan
| | - Navapan Issariyakulkarn
- Division of Gastroenterology King Chulalongkorn Memorial Hospital Thai Red Cross Society Bangkok Thailand
| | - Tanisa Patcharatrakul
- Division of Gastroenterology King Chulalongkorn Memorial Hospital Thai Red Cross Society Bangkok Thailand
- Center of Excellence in Neurogastroenterology and Motility, Department of Medicine, Faculty of Medicine Chulalongkorn University Bangkok Thailand
| | - Katerina Shetler
- Department of Gastroenterology Palo Alto Medical Foundation Mountain View California
| | - Shiko Kuribayashi
- Department of Gastroenterology and Hepatology Gunma University Graduate School of Medicine Maebashi Japan
| | - Toshio Uraoka
- Department of Gastroenterology and Hepatology Gunma University Graduate School of Medicine Maebashi Japan
| | - George Triadafilopoulos
- Division of Gastroenterology and Hepatology Stanford University School of Medicine Stanford California
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McMahan ZH, Khanna D. Managing gastrointestinal complications in patients with systemic sclerosis. ACTA ACUST UNITED AC 2020; 18:531-544. [PMID: 34305387 DOI: 10.1007/s11938-020-00314-x] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022]
Abstract
Objective We sought to critically evaluate the literature published over the past 3 years on the management of gastrointestinal complications in systemic sclerosis (SSc). We emphasize interesting and important new findings to bring the reader up-to-date. We also discuss controversial discoveries and hypotheses currently of interest. Methods We conducted a literature search on PubMed over the last 3 years using the key words "systemic sclerosis," "gastrointestinal," "scleroderma," and "treatment." We also screened clinicaltrials.gov for ongoing trials relevant to the gastrointestinal complications of SSc. Reference lists from recent reviews on the management of gastrointestinal complications of SSc to identify articles that might have been missed in the initial search. Results 103 publications and ongoing clinical trials were identified. We eliminated all case reports and review articles. Ultimately we had 58 articles remaining and we prioritized what we found to be the strongest and/or novel findings to discuss in this review. Conclusions Advances in the management of gastrointestinal disease in SSc continue to evolve. The application of novel therapies and the repurposing of existing therapies for the management of gastrointestinal involvement are shaping the therapeutic arsenal so that we can more effectively manage these complex patients.
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Affiliation(s)
- Z H McMahan
- Johns Hopkins University, Division of Rheumatology, Baltimore, MD, USA
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- University of Michigan, Division of Rheumatology, Ann Arbor, MI, USA
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Abstract
PURPOSE OF REVIEW This review provides important updates in systemic sclerosis (SSc)-related gastrointestinal disease, specifically focusing on the most recent literature. RECENT FINDINGS In the past year, several studies were published that present interesting insights into SSc and gastrointestinal disease. Studies focusing on newly identified risk factors, novel approaches to diagnosis and assessment of disease activity, survival and quality of life demonstrate progress in our understanding of this challenging area. Additional data on specific SSc gastrointestinal-related topics, such as the link between gastrointestinal and pulmonary disease, nutrition, and the microbiome, are also now available. SUMMARY SSc gastrointestinal disease is heterogeneous in its clinical presentation, which presents a challenge in diagnosis and management. In the past year, several studies have evaluated risk factors and clinical features associated with specific gastrointestinal complications in SSc. Objective gastrointestinal testing may help to identify specific SSc gastrointestinal subgroups and provide diagnostic accuracy to guide targeted therapies. Survival in very early SSc is affected by the severity of gastrointestinal involvement. Other important gastrointestinal subsets, including patients with esophageal disease and interstitial lung disease, should carefully be considered when developing a management plan for this patient population.
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