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Reddy S, Patel B, Dellon ES, Eluri S. High prevalence of esophageal motility disorders in patients with rheumatologic diseases. Dis Esophagus 2025; 38:doae108. [PMID: 39586592 DOI: 10.1093/dote/doae108] [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: 07/19/2024] [Revised: 10/25/2024] [Accepted: 11/15/2024] [Indexed: 11/27/2024]
Abstract
While it is commonly known that patients with rheumatologic diseases can have esophageal dysfunction, this association is insufficiently understood. The aim is to determine the prevalence and characteristics of esophageal motility disorders in patients with rheumatic diseases. This is a single-center retrospective study of adults with rheumatologic disease who underwent high-resolution esophageal manometry (HREM). Those with and without a motility disorder (defined per Chicago classification CCv3.0 criteria, given the timing of the prior studies) were compared and multivariable logistic regression was used to determine odds of motility disorder by rheumatic disease. Of 289 patients, the mean age was 60.5 ± 13.8 years. Rheumatic diseases included Raynaud's (42%), rheumatoid arthritis (RA) (39%), Sjogren's (21%), systemic lupus erythematous (19%), systemic sclerosis (17%), and mixed connective tissue disease (13%). On HREM, 58% had an esophageal motility disorder: achalasia (5%), EGJ outflow obstruction (20%), jackhammer (8%), diffuse esophageal spasm (1%), ineffective esophageal motility (28%), and fragmented peristalsis (2%). Of note, 50% of the sample with a normal barium swallow had an esophageal dysmotility disorder on HREM. Those with psoriatic arthritis were less likely to have esophageal dysmotility (73% vs. 27%; P = 0.04). There was decreased odds of esophageal hypocontractility in those with RA (OR [95%CI]: 0.27 [0.12-0.58]) and increased odds (OR [95%CI]: 3.13 [1.16-8.41]) of esophageal hypocontractility among those with scleroderma. Esophageal motor disorders were found in more than half of patients with rheumatologic diseases who underwent HREM. HREM should be considered in patients with rheumatic conditions presenting with esophageal symptoms.
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Affiliation(s)
- Sumana Reddy
- Division of Gastroenterology and Hepatology, University of Texas Southwestern Medical Center, Dallas, TX, USA
| | - Beyla Patel
- Department of Medicine, University of North Carolina School of Medicine, Chapel Hill, NC, USA
| | - Evan S Dellon
- Center for Esophageal Diseases and Swallowing, Division of Gastroenterology and Hepatology, Center for Gastrointestinal Biology and Disease, Chapel Hill, NC, USA
| | - Swathi Eluri
- Center for Esophageal Diseases and Swallowing, Division of Gastroenterology and Hepatology, Center for Gastrointestinal Biology and Disease, Chapel Hill, NC, USA
- Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, FL, USA
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Suresh N, Karanth R, Cheah R, Casey J, Jayne DG, Del Galdo F. Systemic sclerosis and anorectal dysfunction: The Leeds experience. JOURNAL OF SCLERODERMA AND RELATED DISORDERS 2024; 9:210-215. [PMID: 39386265 PMCID: PMC11459476 DOI: 10.1177/23971983241241203] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2023] [Accepted: 02/06/2024] [Indexed: 10/12/2024]
Abstract
Systemic sclerosis is an autoimmune disorder which frequently affects the gastrointestinal tract. Anorectal dysfunction is common in systemic sclerosis and is manifested mainly by atrophy of internal anal sphincter. Faecal incontinence is the result of internal anal sphincter atrophy secondary to systemic sclerosis. In this study, we aimed to assess the internal anal sphincter in 17 patients with faecal incontinence and systemic sclerosis using anorectal manometry and endoanal ultrasound and compare them with an age and gender-matched control group without systemic sclerosis. Most patients have limited cutaneous systemic sclerosis. Majority of the patients with systemic sclerosis and faecal incontinence presented with symptoms of faecal leakage and urgency. Systemic sclerosis patients had low basal sphincter pressures. The mean thickness of internal anal sphincter in systemic sclerosis group was significantly lower than the control group (p < 0.001). Rectal sensation is preserved in systemic sclerosis. There was no difference in the mean thickness of the external anal sphincter between the two groups. To conclude internal anal sphincter is atrophic in systemic sclerosis resulting in decreased resting sphincter pressures and passive faecal leakage. Further investigations and studies are needed to determine the natural course of faecal incontinence in systemic sclerosis, associated risk factors and efficacy of therapeutic interventions.
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Affiliation(s)
- Nikhil Suresh
- Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK
- NIHR Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Chapel Allerton Hospital, Leeds, UK
| | - Ranjitha Karanth
- Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK
- NIHR Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Chapel Allerton Hospital, Leeds, UK
| | - Ramsah Cheah
- NIHR Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Chapel Allerton Hospital, Leeds, UK
| | - John Casey
- NIHR Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Chapel Allerton Hospital, Leeds, UK
| | - David G Jayne
- NIHR Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Chapel Allerton Hospital, Leeds, UK
| | - Francesco Del Galdo
- Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK
- NIHR Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Chapel Allerton Hospital, Leeds, UK
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Cano-García L, Redondo-Rodríguez R, Mena-Vázquez N, Manrique-Arija S, García-Studer A, Ortiz-Marquez F, Borregón-Garrido P, Fernández-Nebro A. Severity and impact of digestive impairment perceived by patients with systemic sclerosis: a cross-sectional study. BMJ Open 2024; 14:e083419. [PMID: 38684244 PMCID: PMC11086412 DOI: 10.1136/bmjopen-2023-083419] [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: 12/19/2023] [Accepted: 04/09/2024] [Indexed: 05/02/2024] Open
Abstract
OBJECTIVES To describe the severity and impact of gastrointestinal involvement in patients with systemic sclerosis (SSc) and identify associated factors. PATIENTS AND METHODS Non-controlled cross-sectional study of patients with SSc (2013 American College of Rheumatology/European League Against Rheumatism criteria). The main variables were severity of gastrointestinal involvement according to the University of California, Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Tract 2.0 instrument (UCLA SCTC GIT 2.0) and dysphagia according to the Eating Assessment Tool-10 (EAT-10). We evaluated reflux, distension, diarrhoea, faecal soilage, constipation, emotional well-being and social functioning, as well as dysphagia. Clinical and epidemiological data were collected using the Mini Nutritional Assessment Short Form (MNA-SF) and the EuroQol-5D-3L. The degree of skin fibrosis was assessed using the modified Rodnan skin score (mRSS). Multivariate models were constructed to analyse factors associated with gastrointestinal involvement and dysphagia. RESULTS Of the 75 patients with SSc included, 58.7% had moderate, severe or very severe reflux, 57.4% had constipation according to UCLA SCTC GIT 2.0 and 49.7% had abdominal distension. Gastrointestinal symptoms interfered significantly with social functioning (42.7%) and emotional well-being (40.0%). Dysphagia (EAT-10≥3) was recorded in 52% of patients, and according to MNA-SF poor nutrition in 30.7%, and clear malnutrition requiring a nutritional intervention in 5.3%. Multivariate adjustment revealed an association between severity of gastrointestinal symptoms according to the mRSS (β=0.249; p=0.002) and Visual Analogue Scale 3-Level EuroQol-5D (VAS-EQ-5D-3L) (β=-0.302; p=0.001), whereas presence of dysphagia was associated with the mRSS (OR=2.794; p=0.015), VAS-EQ-5D-3L (OR=0.950; p=0.005) and malnutrition (MNA-SF≤7; OR=3.920; p=0.041). CONCLUSIONS Patients with SSc frequently present severe gastrointestinal symptoms. These are associated with poor quality of life, more severe skin involvement and malnutrition.
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Affiliation(s)
- Laura Cano-García
- Reumatología, Hospital Regional Universitario de Málaga, Malaga, Spain
- Instituto de Investigación Biomédica de Málaga (IBIMA)-Pataforma BIONAND, Málaga, Spain
| | - Rocío Redondo-Rodríguez
- Reumatología, Hospital Regional Universitario de Málaga, Malaga, Spain
- Instituto de Investigación Biomédica de Málaga (IBIMA)-Pataforma BIONAND, Málaga, Spain
| | - Natalia Mena-Vázquez
- Reumatología, Hospital Regional Universitario de Málaga, Malaga, Spain
- Instituto de Investigación Biomédica de Málaga (IBIMA)-Pataforma BIONAND, Málaga, Spain
| | - Sara Manrique-Arija
- Reumatología, Hospital Regional Universitario de Málaga, Malaga, Spain
- Instituto de Investigación Biomédica de Málaga (IBIMA)-Pataforma BIONAND, Málaga, Spain
- Departamento de Medicina, Universidad de Málaga, Málaga, Spain
| | - Aimara García-Studer
- Reumatología, Hospital Regional Universitario de Málaga, Malaga, Spain
- Instituto de Investigación Biomédica de Málaga (IBIMA)-Pataforma BIONAND, Málaga, Spain
- Departamento de Medicina, Universidad de Málaga, Málaga, Spain
| | - Fernando Ortiz-Marquez
- Reumatología, Hospital Regional Universitario de Málaga, Malaga, Spain
- Instituto de Investigación Biomédica de Málaga (IBIMA)-Pataforma BIONAND, Málaga, Spain
- Departamento de Medicina, Universidad de Málaga, Málaga, Spain
| | - Paula Borregón-Garrido
- Reumatología, Hospital Regional Universitario de Málaga, Malaga, Spain
- Instituto de Investigación Biomédica de Málaga (IBIMA)-Pataforma BIONAND, Málaga, Spain
| | - Antonio Fernández-Nebro
- Reumatología, Hospital Regional Universitario de Málaga, Malaga, Spain
- Instituto de Investigación Biomédica de Málaga (IBIMA)-Pataforma BIONAND, Málaga, Spain
- Departamento de Medicina, Universidad de Málaga, Málaga, Spain
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Bandini G, Alunno A, Ruaro B, Galetti I, Hughes M, McMahan ZH. Significant gastrointestinal unmet needs in patients with Systemic Sclerosis: insights from a large international patient survey. Rheumatology (Oxford) 2024; 63:e92-e93. [PMID: 37725357 PMCID: PMC10907805 DOI: 10.1093/rheumatology/kead486] [Citation(s) in RCA: 6] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2023] [Revised: 07/30/2023] [Accepted: 09/06/2023] [Indexed: 09/21/2023] Open
Affiliation(s)
- Giulia Bandini
- Department of Experimental and Clinical Medicine, Division of Internal Medicine, AOUC, University of Florence, Firenze, Italy
| | - Alessia Alunno
- Department of Life Health and Environmental Sciences, Internal Medicine and Nephrology Division, ASL1 Avezzano-Sulmona-L'Aquila, University of L’Aquila, L'Aquila, Italy
| | - Barbara Ruaro
- Pulmonology Unit, Department of Medical Surgical and Health Sciences, University Hospital of Cattinara, University of Trieste, Trieste, Italy
| | - Ilaria Galetti
- FESCA (Federation of European Scleroderma Associations) Belgium, GILS (Gruppo Italiano, Lotta alla Sclerodermia (Italy), Milan, Italy
| | - Michael Hughes
- Department of Rheumatology, Salford Care Organisation, Northern Care Alliance NHS Foundation Trust, Salford, UK
- Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester, UK
| | - Zsuzsanna H McMahan
- Division of Rheumatology, Department of Medicine, Johns Hopkins University, Baltimore, MD, USA
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von Mühlenbrock C, Madrid AM, Defilippi C, Defilippi C, Soto L. Diffuse Gastrointestinal Motor Compromise in Patients with Scleroderma: Utility of Minimally Invasive Techniques. Dig Dis Sci 2024; 69:191-199. [PMID: 37982941 DOI: 10.1007/s10620-023-08151-5] [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: 05/17/2023] [Accepted: 10/09/2023] [Indexed: 11/21/2023]
Abstract
BACKGROUND Scleroderma is a systemic inflammatory disorder that can compromise the gastrointestinal tract in up to 90% of patients. AIM The purpose of this work is to characterize esophageal, gastric, and intestinal compromise in patients with scleroderma by means of minimally invasive methods and its association with symptoms and severity of their rheumatological condition. METHODS Patients with systemic sclerosis were recruited according to the criteria of the American College of Rheumatology. The study of digestive involvement was carried out on four consecutive days: esophageal manometry was performed on the first day, intestinal manometry on the second day, surface electrogastrography on the third, and hydrogen breath test on the fourth. The Mann-Whitney test was used for quantitative variables and the chi-squared test for categorical variables (p < 0.05). RESULTS A total of 30 patients were included, with an average age of 52.7 years and 93% women. Average disease evolution duration was 6.5 years, 70% with limited variety. Rodnan averaged 12 points, being higher in the diffuse variety. The main symptom was heartburn, followed by abdominal distension, with no differences between subtypes except for diffuse nausea; 80% had intestinal manometric compromise, 76% esophageal manometric compromise, and 30% electrogastrographic compromise. Bacterial overgrowth was evidenced in two-thirds (66%) of the patients, and 23% of the patients had simultaneous esophageal, gastric, and intestinal involvement, which correlated with greater skin involvement but not with gastrointestinal symptoms. CONCLUSIONS Gastrointestinal involvement in patients with scleroderma is frequent and is observed regardless of the symptoms and clinical characteristics of the latter, except for skin involvement.
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Affiliation(s)
- Christian von Mühlenbrock
- Functional Digestive Diseases and Motility Laboratory, Gastroenterology Section, Hospital Clinico Universidad de Chile, Dr. Carlos Lorca 999, Independencia, Santiago, Chile.
- Internal Medicine Department, Gastroenterology Section, Universidad de los Andes, Av San Carlos de Apoquindo 2200, Las Condes, Santiago de Chile, Chile.
| | - Ana María Madrid
- Functional Digestive Diseases and Motility Laboratory, Gastroenterology Section, Hospital Clinico Universidad de Chile, Dr. Carlos Lorca 999, Independencia, Santiago, Chile
| | - Claudia Defilippi
- Functional Digestive Diseases and Motility Laboratory, Gastroenterology Section, Hospital Clinico Universidad de Chile, Dr. Carlos Lorca 999, Independencia, Santiago, Chile
| | - Carlos Defilippi
- Functional Digestive Diseases and Motility Laboratory, Gastroenterology Section, Hospital Clinico Universidad de Chile, Dr. Carlos Lorca 999, Independencia, Santiago, Chile
| | - Lilian Soto
- Rheumatology Section, Department of Medicine, Hospital Clinico Universidad de Chile, Santiago, Chile
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Yang B, Kim BG, Han K, Jung JH, Kim JH, Park DW, Kim SH, Kim EG, Sohn JW, Yoon HJ, Choi H, Lee H. Systemic sclerosis and risk of bronchiectasis: a nationwide longitudinal cohort study. Arthritis Res Ther 2023; 25:209. [PMID: 37872606 PMCID: PMC10591419 DOI: 10.1186/s13075-023-03189-2] [Citation(s) in RCA: 12] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/14/2023] [Accepted: 10/10/2023] [Indexed: 10/25/2023] Open
Abstract
BACKGROUND The association between systemic sclerosis and the development of bronchiectasis is unclear. This study aimed to compare the risk of bronchiectasis between individuals with systemic sclerosis and those without using a nationwide longitudinal dataset. METHODS Using the Korean National Health Insurance Service dataset between 2010 and 2017, we identified 4845 individuals aged ≥ 20 years with systemic sclerosis and 24,225 without systemic sclerosis who were matched 1:5 by age and sex. They were followed up until the date of a bronchiectasis diagnosis, death, or December 31, 2019, whichever came first. RESULTS During a median follow-up period of 6.0 (interquartile range, 3.2-8.7) years, 5.3% of the systemic sclerosis cohort and 1.9% of the matched cohort developed bronchiectasis, with incidence rates of 9.99 and 3.23 per 1000 person-years, respectively. Even after adjusting for potential confounders, the risk of incident bronchiectasis was significantly higher in the systemic sclerosis cohort than in the matched cohort (adjusted hazard ratio 2.63, 95% confidence interval 2.22-3.12). A subgroup analysis of individuals with systemic sclerosis revealed that the risk of incident bronchiectasis was notably higher in younger individuals aged 20-39 years (P for interaction = 0.048) and in those without other coexisting connective tissue diseases (P for interaction = 0.006) than in their counterparts. CONCLUSIONS The risk of incident bronchiectasis is higher in individuals with systemic sclerosis than those without. Bronchiectasis should be considered one of the pulmonary manifestations related to systemic sclerosis.
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Affiliation(s)
- Bumhee Yang
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju, Republic of Korea
| | - Bo-Guen Kim
- Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University College of Medicine, 222-1, Wangsimni-ro, Seoul, Seongdong-gu, 04763, Republic of Korea
| | - Kyungdo Han
- Department of Statistics and Actuarial Science, Soongsil University, Seoul, Republic of Korea
| | - Jin-Hyung Jung
- Department of Biostatistics, College of Medicine, Catholic University of Korea, Seoul, Republic of Korea
| | - Ji Hyoun Kim
- Division of Rheumatology, Department of Internal Medicine, Chungbuk National University Hospital, Cheongju, Republic of Korea
| | - Dong Won Park
- Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University College of Medicine, 222-1, Wangsimni-ro, Seoul, Seongdong-gu, 04763, Republic of Korea
| | - Sang-Heon Kim
- Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University College of Medicine, 222-1, Wangsimni-ro, Seoul, Seongdong-gu, 04763, Republic of Korea
| | - Eung-Gook Kim
- Department of Biochemistry, Chungbuk National University College of Medicine, Cheongju, Republic of Korea
| | - Jang Won Sohn
- Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University College of Medicine, 222-1, Wangsimni-ro, Seoul, Seongdong-gu, 04763, Republic of Korea
| | - Ho Joo Yoon
- Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University College of Medicine, 222-1, Wangsimni-ro, Seoul, Seongdong-gu, 04763, Republic of Korea
| | - Hayoung Choi
- Division of Pulmonary, Allergy, and Critical Care Medicine, Department of Internal Medicine, Hallym University Kangnam Sacred Heart Hospital, Hallym University College of Medicine, 1 Singil-ro, Seoul, Yeongdeungpo-gu, 07441, Republic of Korea.
| | - Hyun Lee
- Division of Pulmonary Medicine and Allergy, Department of Internal Medicine, Hanyang University College of Medicine, 222-1, Wangsimni-ro, Seoul, Seongdong-gu, 04763, Republic of Korea.
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Tucker AE, Perin J, Volkmann ER, Abdi T, Shah AA, Pandolfino J, Silver RM, McMahan ZH. Associations Between Patterns of Esophageal Dysmotility and Extra-Intestinal Features in Patients With Systemic Sclerosis. Arthritis Care Res (Hoboken) 2023; 75:1715-1724. [PMID: 36576023 PMCID: PMC11019887 DOI: 10.1002/acr.25080] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2022] [Revised: 11/29/2022] [Accepted: 12/20/2022] [Indexed: 12/29/2022]
Abstract
OBJECTIVE The gastrointestinal tract is commonly involved in patients with systemic sclerosis (SSc) with varied manifestations. As our understanding of SSc gastrointestinal disease pathogenesis and risk stratification is limited, we sought to investigate whether patterns of esophageal dysfunction associate with specific clinical phenotypes in SSc. METHODS Patients enrolled in the Johns Hopkins Scleroderma Center Research Registry who completed high-resolution esophageal manometry (HREM) studies as part of their clinical care between 2011 and 2020 were identified. Associations between esophageal abnormalities on HREM (absent contractility [AC], ineffective esophageal motility [IEM], hypotensive lower esophageal sphincter [hypoLES]) and patient demographic information, clinical characteristics, and autoantibody profiles were examined. RESULTS Ninety-five patients with SSc had HREM data. Sixty-five patients (68.4%) had AC (37 patients with only AC, 28 patients with AC and a hypoLES), 9 patients (9.5%) had IEM, and 11 patients (11.6%) had normal studies. AC was significantly associated with diffuse cutaneous disease (38.5% versus 10.0%; P < 0.01), more severe Raynaud's phenomenon, including digital pits, ulcers, or gangrene (56.9% versus 30.0%; P = 0.02), and reduced median diffusing capacity of lung for carbon monoxide (50.6% versus 72.2%; P = 0.03). AC was observed in most of the patients who died (13 of 14; P = 0.06). These findings were not seen in patients with IEM. CONCLUSION Among patients with SSc, AC is associated with a significantly more severe clinical phenotype. IEM may associate with a milder phenotype. Further studies are needed to evaluate AC, IEM, and their clinical impact relative to the timing of other end-organ complications in SSc.
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Affiliation(s)
- Ana E Tucker
- Medical University of South Carolina, Charleston
| | - Jamie Perin
- Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland
| | | | - Tsion Abdi
- Johns Hopkins University, Baltimore, Maryland
| | - Ami A Shah
- Johns Hopkins University, Baltimore, Maryland
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Chatterjee S, Elsheikh TM, Kirby DF. Esophageal Plaques in a 68-Year-Old Woman With Systemic Sclerosis. Am J Med 2023:S0002-9343(23)00255-3. [PMID: 37068577 DOI: 10.1016/j.amjmed.2023.03.032] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/19/2023] [Accepted: 03/20/2023] [Indexed: 04/19/2023]
Affiliation(s)
- Soumya Chatterjee
- Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Staff, Department of Rheumatic and Immunologic Diseases, Orthopedic and Rheumatologic Institute, Cleveland Clinic, Cleveland, Ohio 44195, U.S.A..
| | - Tarik M Elsheikh
- Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio 44195, U.S.A
| | - Donald F Kirby
- Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Director, Center for Human Nutrition, Department of Gastroenterology, Hepatology & Nutrition, Medical Director, Intestinal Transplant, Digestive Disease and Surgery Institute, Cleveland Clinic
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Chatterjee S, Perelas A, Yadav R, Kirby DF, Singh A. Viewpoint: a multidisciplinary approach to the assessment of patients with systemic sclerosis-associated interstitial lung disease. Clin Rheumatol 2023; 42:653-661. [PMID: 36271064 PMCID: PMC9935731 DOI: 10.1007/s10067-022-06408-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/06/2022] [Revised: 10/05/2022] [Accepted: 10/07/2022] [Indexed: 11/03/2022]
Abstract
Systemic sclerosis (SSc) is a rare and heterogeneous disease affecting the skin and internal organs. SSc-associated ILD (SSc-ILD) is a common and often early manifestation of SSc. This article discusses the rationale for a multidisciplinary approach (MDA) to the early identification and assessment of patients with SSc-ILD. Diagnosis of SSc-ILD is often challenging as patients with early disease can be asymptomatic, and SSc-ILD symptoms, such as exertional dyspnea and cough, are non-specific. High-resolution computed tomography (HRCT) of the lungs is the gold standard for diagnosis of SSc-ILD since pulmonary function tests lack sensitivity and specificity, especially in early disease. On HRCT, most patients with SSc-ILD have a non-specific interstitial pneumonia (NSIP) pattern. In addition, findings of pulmonary hypertension and esophageal dysmotility may be present. The multi-organ involvement of SSc and the diverse spectrum of symptoms support an MDA for the diagnosis and assessment of patients with SSc-ILD, with input from rheumatologists, pulmonologists, gastroenterologists, radiologists, and other specialists. Key Points • Interstitial lung disease (ILD) is a common manifestation of systemic sclerosis (SSc). • Early diagnosis is key to reducing the morbidity and mortality associated with SSc-ILD and other manifestations of SSc. • The multi-organ involvement of SSc supports a multidisciplinary approach to the diagnosis and assessment of patients with SSc-ILD, with input from rheumatologists, pulmonologists, gastroenterologists, radiologists, and other specialists.
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Affiliation(s)
- Soumya Chatterjee
- Department of Rheumatic and Immunologic Diseases, Orthopedic and Rheumatology Institute, Cleveland Clinic, Cleveland, OH USA
| | - Apostolos Perelas
- Department of Pulmonary and Critical Care Medicine, Virginia Commonwealth University, Richmond, VA USA
| | - Ruchi Yadav
- Department of Diagnostic Radiology, Imaging Institute, Cleveland Clinic, Cleveland, OH USA
| | - Donald F. Kirby
- Department of Gastroenterology, Hepatology, and Nutrition, Center for Human Nutrition, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, OH USA
| | - Amandeep Singh
- Department of Gastroenterology, Hepatology, and Nutrition, Center for Human Nutrition, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, OH USA
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Liu P, Chai J, Dai L, Chen B, Zhao J, Lu M, Zeng L, Xia Z, Mu R. Development of a Diagnostic Model Focusing on Esophageal Dysmotility in Patients with Systemic Sclerosis. Diagnostics (Basel) 2022; 12:diagnostics12123142. [PMID: 36553149 PMCID: PMC9776849 DOI: 10.3390/diagnostics12123142] [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] [Received: 11/10/2022] [Revised: 12/06/2022] [Accepted: 12/11/2022] [Indexed: 12/15/2022] Open
Abstract
OBJECTIVE Esophageal dysmotility is a common and neglected complication of systemic sclerosis (SSc) associated with poor prognosis, while the assessment remains a challenge. We aimed to develop a diagnostic model for esophageal dysmotility in SSc patients that provides individualized risk estimates. METHODS Seventy-five SSc patients who underwent high-resolution manometry (HRM) were included in the study. Esophageal widest diameter (WED) was measured on a chest CT scan. Esophageal parameters between patients with and without esophageal dysmotility were compared. Multivariate logistic regression analysis and Least Absolute Shrinkage and Selection Operator (LASSO) regression were used to fit the model. The diagnostic model was evaluated by discrimination and calibration. Internal validation was estimated using the enhanced bootstrap method with 1000 repetitions. RESULTS Sixty-one systemic sclerosis patients (81.3%) were diagnosed with esophageal dysmotility according to the Chicago Classification v 3.0. The diagnostic model for evaluating the probability of esophageal dysmotility integrated clinical and imaging features, including disease duration, ILD, and WED. The model displayed good discrimination with an area under the curve (AUC) of 0.923 (95% CI: 0.837-1.000), a Brier score of 0.083, and good calibration. A high AUC value of 0.911 could still be achieved in the internal validation. CONCLUSION The diagnostic model, which combines the disease duration, ILD, and imaging feature (WED), is an effective and noninvasive method for predicting esophageal dysmotility in SSc patients.
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Affiliation(s)
- Peiling Liu
- Department of Rheumatology and Immunology, Peking University Third Hospital, No. 49 Huayuan North Road, Beijing 100191, China
| | - Jing Chai
- Department of Rheumatology and Immunology, Peking University Third Hospital, No. 49 Huayuan North Road, Beijing 100191, China
| | - Liyi Dai
- Department of Rheumatology and Immunology, Peking University Third Hospital, No. 49 Huayuan North Road, Beijing 100191, China
| | - Beidi Chen
- Department of Rheumatology and Immunology, Peking University Third Hospital, No. 49 Huayuan North Road, Beijing 100191, China
| | - Jinxia Zhao
- Department of Rheumatology and Immunology, Peking University Third Hospital, No. 49 Huayuan North Road, Beijing 100191, China
| | - Ming Lu
- Department of Respiratory and Critical Care Medicine, Peking University Third Hospital, Beijing 100191, China
- Department of Infectious Diseases, Peking University Third Hospital, Beijing 100191, China
| | - Lin Zeng
- Research Center of Clinical Epidemiology, Peking University Third Hospital, Beijing 100191, China
| | - Zhiwei Xia
- Department of Gastroenterology, Peking University Third Hospital, Beijing 100191, China
| | - Rong Mu
- Department of Rheumatology and Immunology, Peking University Third Hospital, No. 49 Huayuan North Road, Beijing 100191, China
- Correspondence:
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Rinaldi S, Balsillie C, Truchon C, Al-Mubarak A, Mura M, Madill J. Nutrition implications of intrinsic restrictive lung disease. Nutr Clin Pract 2022; 37:239-255. [PMID: 35253924 DOI: 10.1002/ncp.10849] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2021] [Revised: 01/26/2022] [Accepted: 01/28/2022] [Indexed: 01/03/2025] Open
Abstract
Restrictive lung disease is defined as a reduction in lung volume that may be due to intraparenchymal or extraparenchymal causes. Intraparenchymal causes falls under the umbrella term of interstitial lung disease (ILD) and includes idiopathic pulmonary fibrosis. This manuscript provides an overview of ILD and can be beneficial for all clinicians working with patients with ILD. Although not well documented, the prevalence of malnutrition in patients with ILD has been reported to be between ~9% and 55%. Body mass index has been shown to predict survival; but more recently, research has suggested that fat-free mass has a larger influence on survival. There is insufficient evidence to support the use of antioxidant or vitamin supplementation to help diminish the chronic inflammatory process that is seen in this patient population. There are data from studies examining the vitamin D status in this patient population, but research on vitamin D supplementation appears to be lacking. Registered dietitian nutritionists should continue to advocate and play a more prominent role in the nutrition management of patients with ILD as part of standard of care.
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Affiliation(s)
- Sylvia Rinaldi
- School of Food and Nutritional Science, Brescia University College, Western University, London, Ontario, Canada
| | - Christine Balsillie
- School of Food and Nutritional Science, Brescia University College, Western University, London, Ontario, Canada
| | - Cassandra Truchon
- School of Food and Nutritional Science, Brescia University College, Western University, London, Ontario, Canada
| | - Awatif Al-Mubarak
- School of Food and Nutritional Science, Brescia University College, Western University, London, Ontario, Canada
| | - Marco Mura
- Division of Respirology, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada
| | - Janet Madill
- School of Food and Nutritional Science, Brescia University College, Western University, London, Ontario, Canada
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McMahan ZH, Tucker AE, Perin J, Volkmann ER, Kulkarni S, Ziessman HA, Pasricha PJ, Wigley FM. Relationship Between Gastrointestinal Transit, Medsger Gastrointestinal Severity, and University of California-Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 Symptoms in Patients With Systemic Sclerosis. Arthritis Care Res (Hoboken) 2022; 74:442-450. [PMID: 33064934 PMCID: PMC8050123 DOI: 10.1002/acr.24488] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2020] [Revised: 09/22/2020] [Accepted: 10/13/2020] [Indexed: 12/21/2022]
Abstract
OBJECTIVE Systemic sclerosis (SSc)-associated gastrointestinal (GI) complications are attributed to a variety of factors, including diet, microbiota dysbiosis, or GI transit abnormalities. Our objective was to examine the contribution of abnormal GI transit to SSc Medsger GI severity scores and/or University of California Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract (UCLA GIT) 2.0 symptoms. METHODS Patients with SSc and GI symptoms (n = 71) and healthy controls (n = 18) underwent whole gut transit (WGT) scintigraphy to assess transit from the esophagus to the colon. The presence of delayed transit and percent emptying in each GI region were measured. We compared the WGT measurements between categories of the Medsger GI severity score (0-4) and across UCLA GIT 2.0 domains and total score (0-3). RESULTS A total of 88% of patients had >1 abnormal region of the gut on WGT scintigraphy. All patients requiring total parenteral nutrition had delayed small bowel transit, compared to only approximately 11% of patients in other Medsger GI severity groups (P ≤ 0.01). Severe colonic transit delays were more likely in patients with Medsger GI scores of 3 (pseudo-obstruction and/or malabsorption) compared to other Medsger GI groups (P = 0.02). Seventy-percent of these patients had ≤30% colonic emptying at 72 hours. Modest associations were noted between gastroesophageal reflux disease symptoms and delayed esophageal (r = -0.31, P = 0.05) and gastric emptying (r = -0.32, P = 0.05). CONCLUSION These data are important in providing evidence that SSc bowel disease affects transit of GI content and that delay in transit accounts in part for both bowel symptoms and Medsger GI severity. Prospective studies examining the benefit of early therapeutic intervention targeting GI transit abnormalities in patients at high risk for severe GI complications are needed.
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Affiliation(s)
| | - Ana E Tucker
- Johns Hopkins Bayview Medical Center, Baltimore, Maryland
| | - Jamie Perin
- Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland
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Kondoh Y, Makino S, Ogura T, Suda T, Tomioka H, Amano H, Anraku M, Enomoto N, Fujii T, Fujisawa T, Gono T, Harigai M, Ichiyasu H, Inoue Y, Johkoh T, Kameda H, Kataoka K, Katsumata Y, Kawaguchi Y, Kawakami A, Kitamura H, Kitamura N, Koga T, Kurasawa K, Nakamura Y, Nakashima R, Nishioka Y, Nishiyama O, Okamoto M, Sakai F, Sakamoto S, Sato S, Shimizu T, Takayanagi N, Takei R, Takemura T, Takeuchi T, Toyoda Y, Yamada H, Yamakawa H, Yamano Y, Yamasaki Y, Kuwana M. 2020 guide for the diagnosis and treatment of interstitial lung disease associated with connective tissue disease. Respir Investig 2021; 59:709-740. [PMID: 34602377 DOI: 10.1016/j.resinv.2021.04.011] [Citation(s) in RCA: 54] [Impact Index Per Article: 13.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2021] [Revised: 04/20/2021] [Accepted: 04/21/2021] [Indexed: 01/29/2023]
Abstract
The prognosis of patients with connective tissue disease (CTD) has improved significantly in recent years, but interstitial lung disease (ILD) associated with connective tissue disease (CTD-ILD) remains a refractory condition, which is a leading cause of mortality. Because it is an important prognostic factor, many observational and interventional studies have been conducted to date. However, CTD is a heterogeneous group of conditions, which makes the clinical course, treatment responses, and prognosis of CTD-ILD extremely diverse. To summarize the current understanding and unsolved questions, the Japanese Respiratory Society and the Japan College of Rheumatology collaborated to publish the world's first guide focusing on CTD-ILD, based on the evidence and expert consensus of pulmonologists and rheumatologists, along with radiologists, pathologists, and dermatologists. The task force members proposed a total of 27 items, including 7 for general topics, 9 for disease-specific topics, 3 for complications, 4 for pharmacologic treatments, and 4 for non-pharmacologic therapies, with teams of 2-4 authors and reviewers for each item to prepare a consensus statement based on a systematic literature review. Subsequently, public opinions were collected from members of both societies, and a critical review was conducted by external reviewers. Finally, the task force finalized the guide upon discussion and consensus generation. This guide is expected to contribute to the standardization of CTD-ILD medical care and is also useful as a tool for promoting future research by clarifying unresolved issues.
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Affiliation(s)
- Yasuhiro Kondoh
- Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
| | - Shigeki Makino
- Rheumatology Division, Osaka Medical College Mishima-Minami Hospital, Takatsuki, Osaka, Japan
| | - Takashi Ogura
- Division of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Kanagawa, Japan
| | - Takafumi Suda
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan
| | - Hiromi Tomioka
- Department of Respiratory Medicine, Kobe City Medical Center West Hospital, Kobe, Hyogo, Japan
| | - Hirofumi Amano
- Department of Internal Medicine and Rheumatology, Juntendo University Graduate School of Medicine, Bunkyo, Tokyo, Japan
| | - Masaki Anraku
- Department of Thoracic Surgery, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Itabashi, Tokyo, Japan
| | - Noriyuki Enomoto
- Health Administration Center, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan
| | - Takao Fujii
- Department of Rheumatology and Clinical Immunology, Wakayama Medical University, Wakayama, Wakayama, Japan
| | - Tomoyuki Fujisawa
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan
| | - Takahisa Gono
- Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Bunkyo, Tokyo, Japan
| | - Masayoshi Harigai
- Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Shinjuku, Tokyo, Japan
| | - Hidenori Ichiyasu
- Department of Respiratory Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Kumamoto, Japan
| | - Yoshikazu Inoue
- Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
| | - Takeshi Johkoh
- Department of Radiology, Kansai Rosai Hospital, Amagasaki, Hyogo, Japan
| | - Hideto Kameda
- Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Toho University, Meguro, Tokyo, Japan
| | - Kensuke Kataoka
- Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan
| | - Yasuhiro Katsumata
- Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Shinjuku, Tokyo, Japan
| | - Yasushi Kawaguchi
- Department of Rheumatology, Tokyo Women's Medical University School of Medicine, Shinjuku, Tokyo, Japan
| | - Atsushi Kawakami
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan
| | - Hideya Kitamura
- Division of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Kanagawa, Japan
| | - Noboru Kitamura
- Division of Hematology and Rheumatology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan
| | - Tomohiro Koga
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan
| | - Kazuhiro Kurasawa
- Department of Rheumatology, Dokkyo Medical University, Mibu, Tochigi, Japan
| | - Yutaro Nakamura
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan
| | - Ran Nakashima
- Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Sakyo, Kyoto, Japan
| | - Yasuhiko Nishioka
- Department of Respiratory Medicine and Rheumatology, Graduate School of Biomedical Sciences, Tokushima University, Tokushima, Tokushima, Japan
| | - Osamu Nishiyama
- Department of Respiratory Medicine and Allergology, Kindai University Faculty of Medicine, Osakasayama, Osaka, Japan
| | - Masaki Okamoto
- Department of Respirology, National Hospital Organization Kyushu Medical Center, Fukuoka, Fukuoka, Japan
| | - Fumikazu Sakai
- Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, Hidaka, Saitama, Japan
| | - Susumu Sakamoto
- Department of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
| | - Shinji Sato
- Division of Rheumatology, Department of Internal Medicine, Tokai University School of Medicine, Isehara, Kanagawa, Japan
| | - Toshimasa Shimizu
- Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Nagasaki, Japan
| | - Noboru Takayanagi
- Department of Respiratory Medicine, Saitama Cardiovascular and Respiratory Center, Kumagaya, Saitama, Japan
| | - Reoto Takei
- Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan
| | - Tamiko Takemura
- Department of Pathology, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Kanagawa, Japan
| | - Tohru Takeuchi
- Department of Internal Medicine (IV), Osaka Medical and Pharmaceutical University, Takatsuki, Osaka, Japan
| | - Yuko Toyoda
- Department of Respiratory Medicine, Japanese Red Cross Kochi Hospital, Kochi, Kochi, Japan
| | - Hidehiro Yamada
- Center for Rheumatic Diseases, Seirei Yokohama Hospital, Yokohama, Kanagawa, Japan
| | - Hideaki Yamakawa
- Department of Respiratory Medicine, Saitama Red Cross Hospital, Saitama, Saitama, Japan
| | - Yasuhiko Yamano
- Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan
| | - Yoshioki Yamasaki
- Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Bunkyo, Tokyo, Japan
| | - Masataka Kuwana
- Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Bunkyo, Tokyo, Japan
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Luquez-Mindiola A, Atuesta AJ, Gómez-Aldana AJ. Gastrointestinal manifestations of systemic sclerosis: An updated review. World J Clin Cases 2021; 9:6201-6217. [PMID: 34434988 PMCID: PMC8362561 DOI: 10.12998/wjcc.v9.i22.6201] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/18/2021] [Revised: 03/30/2021] [Accepted: 06/07/2021] [Indexed: 02/06/2023] Open
Abstract
Systemic sclerosis is an autoimmune disease characterized by vascular disease, fibrosis of the skin, and internal organ dysfunction. Gastrointestinal involvement is the most frequent complication of internal organs, impacting up to 90% of patients. Gastrointestinal involvement can affect any region of the gastrointestinal tract from the mouth to the anus, with a predominance of disorders being observed at the level of the upper digestive tract. The gastrointestinal involvement primarily involves the esophagus, small bowel, and rectum. The severity of gastrointestinal involvement affects quality of life and is a marker of worse prognosis and mortality in these patients. In this review, we describe the current findings regarding gastrointestinal involvement by this entity.
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Affiliation(s)
| | - Alexis Javier Atuesta
- Department of Internal Medicine, Universidad Nacional de Colombia, Bogota 11711, Colombia
| | - Andres Jose Gómez-Aldana
- Department of Endoscopy, Santa Fe Foundation of Bogotá (Fundación Santa Fe de Bogotá), Bogotá 11711, Colombia
- Faculty of Medicine, Universidad de los Andes, Bogota 11711, Colombia
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Review for best practice in clinical rheumatology juvenile systemic sclerosis - Updates and practice points. Best Pract Res Clin Rheumatol 2021; 35:101688. [PMID: 33896752 DOI: 10.1016/j.berh.2021.101688] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
Juvenile systemic sclerosis (jSSc) is a rare, severe autoimmune disease associated with life-threatening multiorgan inflammatory-driven fibrosis. Recognition early in the disease process, when treatment is more effective, is critical. We outline insights from the authors, who specialize and host jSSc cohorts, combined with recent literature review combining available juvenile-onset and applicable adult-onset studies regarding SSc evaluation, which can be extrapolated to children. Practice tips are provided for each main organ system.
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Loganathan P, Gajendran M, Davis B, McCallum R. Efficacy and Safety of Robotic Dor Fundoplication on Severe Gastroesophageal Reflux Disease in Patients With Scleroderma. J Investig Med High Impact Case Rep 2021; 9:23247096211051211. [PMID: 34654321 PMCID: PMC8521723 DOI: 10.1177/23247096211051211] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2021] [Revised: 08/19/2021] [Accepted: 09/16/2021] [Indexed: 12/02/2022] Open
Abstract
Systemic sclerosis (SSc) is a disease that affects the gastrointestinal tract resulting in its atrophy and fibrosis of smooth muscles. Approximately 80% of SSc patients develop both gastroesophageal reflux disease (GERD) and dysphagia. The nocturnal GERD can cause regurgitation and aspiration, which can further aggravate the pulmonary fibrosis from SSc. Also, their dysphagia is further worsened by performing standard Nissen fundoplication. Therefore, we aimed to investigate whether Dor fundoplication (a 180° anterior wrap) can reduce nocturnal heartburn and regurgitation without worsening dysphagia in patients with SSc and severe GERD. Five SSc patients with drug-refractory severe GERD underwent a Dor fundoplication procedure with a median follow-up of 2 years (range: 1-5 years). In all 5 patients, the preoperative high-resolution manometry showed significant impairment of esophageal motility. Patients were interviewed postoperatively to assess for nocturnal and diurnal GERD symptoms, treatment response, the status of dysphagia, and adverse effects of surgery. The average age of 5 patients was 50 years and all were females. Four of the 5 patients (80%) reported 90% improvement in both diurnal and nocturnal GERD symptoms since surgery, with no nocturnal reflux, heartburn, or regurgitation, and reports to sleep at night without requiring any more pillows or wedges. About 50% of patients reported a decrease in their proton pump inhibitor dosage after surgery compared to before surgery. No surgical complication was reported and specifically, no worsening of dysphagia. The Dor fundoplication performed for refractory GERD in SSc patients substantially decreases heartburn and regurgitation, primarily nocturnal, without affecting dysphagia, thus improving the quality of life.
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Affiliation(s)
| | - Mahesh Gajendran
- Texas Tech University Health Sciences Center El Paso, Texas
- UT Health San Antonio, TX, USA
| | - Brian Davis
- Texas Tech University Health Sciences Center El Paso, Texas
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Natsui K, Tsuchiya A, Terai S. Refractory hemorrhagic esophageal ulcers by Candida esophagitis with advanced systemic sclerosis. JGH OPEN 2020; 4:1007-1008. [PMID: 33102777 PMCID: PMC7578314 DOI: 10.1002/jgh3.12353] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 04/02/2020] [Revised: 04/25/2020] [Accepted: 04/27/2020] [Indexed: 12/18/2022]
Abstract
A 64-year-old woman diagnosed with rheumatoid arthritis (RA) and systemic sclerosis (SSc) was admitted to our hospital with chief complaints of uncontrolled bleeding from esophageal ulcers and an inability to consume meals. For RA and SSc, she was treated with prednisolone and abatacept and was taking vonoprazan as prophylaxis for steroid-induced gastric ulcers. She was diagnosed with severe Candida esophagitis, with multiple large and small ulcers with bleeding, based on esophagogastroduodenoscopy and pathological findings. We performed comprehensive treatment; abatacept was discontinued, and total parenteral nutrition was initiated along with antifungal therapy. Improvement in the esophageal ulcers was observed. Although severe Candida esophagitis is a rare condition, we should keep in mind that severe Candida esophagitis can occur in patients with an immunosuppressive compromised host and esophageal movement disorders such as SSc. Regular follow up by endoscopy and prophylactic treatment to prevent severe esophagitis may be necessary.
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Affiliation(s)
- Kazuki Natsui
- Division of Gastroenterology and Hepatology Graduate School of Medical and Dental Sciences, Niigata University Niigata Japan
| | - Atsunori Tsuchiya
- Division of Gastroenterology and Hepatology Graduate School of Medical and Dental Sciences, Niigata University Niigata Japan
| | - Shuji Terai
- Division of Gastroenterology and Hepatology Graduate School of Medical and Dental Sciences, Niigata University Niigata Japan
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Alam F, Abdulaziz HM, Ul Haq I, Mahdy SM, Mohammed Siam AR, Chandra P, Al Emadi S. Characteristics of patients with systemic sclerosis living in Qatar. Qatar Med J 2020; 2019:16. [PMID: 31903322 PMCID: PMC6929649 DOI: 10.5339/qmj.2019.16] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2019] [Accepted: 07/18/2019] [Indexed: 11/29/2022] Open
Abstract
Objective: The aim of this study was to determine the demographic, clinical, and immunological characteristics of patients with systemic sclerosis living in Qatar. Method: This retrospective study included 42 patients with systemic sclerosis who attended Rheumatology Clinics at Hamad General Hospital in Doha, Qatar, between January 2000 and December 2014. All patients fulfilled the 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis. Results: The 42 consecutively recruited patients of mixed ethnicities consisted of 37 (88.1%) females and 5 (11.9%) males. Of the total 42 patients, 22 (52.4%) had diffuse cutaneous systemic sclerosis (dcSSc) and 20 (47.6%) had limited cutaneous systemic sclerosis (lcSSc). Mean age at onset of first symptoms was 34.5 ± 12 years, and mean age at diagnosis was 36.1 ± 11.5 years. During follow-up, Raynaud's phenomenon occurred in 36 (85.7%) patients, sclerodactyly in 39 (92.9%) patients, digital ulcers in 16 (38.1%) patients, calcinosis in 6 (14.3%) patients, telangiectasia in 16 (38.1%) patients, and arthritis in 13 (31%) patients. The gastrointestinal and respiratory systems were the most frequently affected internal organs. Gastrointestinal involvement was present in 36 (85.7%) patients, and respiratory involvement was found in 30 (71.4%) patients. The majority of patients had positive antinuclear antibodies (ANA; 97.6%). Anti-Scl-70 antibody was found in 66.7% and anti-centromere antibody (ACA) was detected in 14.3% of the patients. Conclusion: To our knowledge, this is the first study that describes the clinical and immunological profile of patients with systemic sclerosis living in Qatar. This study cohort showed an earlier age of disease onset and diagnosis than that reported in other international studies. Furthermore, in contrast to several other studies, the diffuse type of scleroderma was more commonly observed than the limited type, which resulted in a high frequency of anti-Scl-70 antibody and interstitial lung disease.
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Affiliation(s)
- Fiaz Alam
- Rheumatology Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar
| | | | - Irfan Ul Haq
- Pulmonary Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar
| | - Salah Mohamed Mahdy
- Rheumatology Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar
| | | | - Prem Chandra
- Medical Research Centre, Hamad Medical Corporation, Doha, Qatar
| | - Samar Al Emadi
- Rheumatology Section, Department of Medicine, Hamad Medical Corporation, Doha, Qatar
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20
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Chwiesko A, Kowal-Bielecka O, Sierakowski S. Perspectives on the interlinked nature of systemic sclerosis and reflux disease. Expert Rev Gastroenterol Hepatol 2019; 13:213-227. [PMID: 30791766 DOI: 10.1080/17474124.2019.1561274] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Systemic sclerosis (SSc) is a multisystem connective tissue disease, characterized by chronic inflammation and vascular changes that result in esophageal smooth muscle atrophy and fibrosis. Subsequent progressive loss of peristalsis in the distal esophagus and loss of lower esophageal sphincter function lead to problems with the protective barrier and exposure of sensitive tissues to the gastroduodenal contents, a disorder called reflux disease. Areas covered: Depending on the range, nature and symptoms of the disease, the term 'reflux disease' may refer to gastroesophageal reflux, laryngopharyngeal reflux, microaspiration into the airways and silent reflux. Despite the links between these visceral complications, this connection remains controversial. This is due to a lack of complete understanding, the asymptomatic nature of the disease and the limited diagnostic accuracy of tests, which can delay diagnosis. Such delays are problematic, given that the early detection of GERD in SSc patients, the timing of assessment, the treatment of the organs involved are critical aspects of patient prognosis and disease outcome. Expert commentary: This review summarizes the most recent knowledge about the pathophysiology, diagnosis and prospective treatment of GERD in SSc patients and highlights how innovative technologies applied through an integrative, interdisciplinary approach may soon lead to effective treatment strategies.
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Affiliation(s)
- Adam Chwiesko
- a Department of Gastroenterology and Internal Medicine , Medical University of Bialystok , Bialystok , Poland
| | - Otylia Kowal-Bielecka
- b Department of Rheumatology and Internal Medicine , Medical University of Bialystok , Bialystok , Poland
| | - Stanislaw Sierakowski
- b Department of Rheumatology and Internal Medicine , Medical University of Bialystok , Bialystok , Poland
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Asano Y, Jinnin M, Kawaguchi Y, Kuwana M, Goto D, Sato S, Takehara K, Hatano M, Fujimoto M, Mugii N, Ihn H. Diagnostic criteria, severity classification and guidelines of systemic sclerosis. J Dermatol 2018; 45:633-691. [PMID: 29687465 DOI: 10.1111/1346-8138.14162] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2017] [Accepted: 11/06/2017] [Indexed: 01/17/2023]
Abstract
Several effective drugs have been identified for the treatment of systemic sclerosis (SSc). However, in advanced cases, not only their effectiveness is reduced but they may be also harmful due to their side-effects. Therefore, early diagnosis and early treatment is most important for the treatment of SSc. We established diagnostic criteria for SSc in 2003 and early diagnostic criteria for SSc in 2011, for the purpose of developing evaluation of each organ in SSc. Moreover, in November 2013, the American College of Rheumatology and the European Rheumatology Association jointly developed new diagnostic criteria for increasing their sensitivity and specificity, so we revised our diagnostic criteria and severity classification of SSc. Furthermore, we have revised the clinical guideline based on the newest evidence. In particular, the clinical guideline was established by clinical questions based on evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guideline easy to use and reliable based on the newest evidence, and to present guidance as specific as possible for various clinical problems in treatment of SSc.
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Affiliation(s)
- Yoshihide Asano
- Department of Dermatology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan
| | - Masatoshi Jinnin
- Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
| | - Yasushi Kawaguchi
- Institute of Rheumatology, Tokyo Woman's Medical University, Tokyo, Japan
| | - Masataka Kuwana
- Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan
| | - Daisuke Goto
- Department of Rheumatology, Faculty of Medicine, Univertity of Tsukuba, Ibaraki, Japan
| | - Shinichi Sato
- Department of Dermatology, Faculty of Medicine, The University of Tokyo, Tokyo, Japan
| | - Kazuhiko Takehara
- Department of Molecular Pathology of Skin, Faculty of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Ishikawa, Japan
| | - Masaru Hatano
- Graduate School of Medicine Department of Therapeutic Strategy for Heart Failure, The University of Tokyo, Tokyo, Japan
| | - Manabu Fujimoto
- Department of Dermatology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan
| | - Naoki Mugii
- Section of Rehabilitation, Kanazawa University Hospital, Ishikawa, Japan
| | - Hironobu Ihn
- Department of Dermatology and Plastic Surgery, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan
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McFarlane IM, Bhamra MS, Kreps A, Iqbal S, Al-Ani F, Saladini-Aponte C, Grant C, Singh S, Awwal K, Koci K, Saperstein Y, Arroyo-Mercado FM, Laskar DB, Atluri P. Gastrointestinal Manifestations of Systemic Sclerosis. ACTA ACUST UNITED AC 2018; 8. [PMID: 30057856 PMCID: PMC6059963 DOI: 10.4172/2161-1149.1000235] [Citation(s) in RCA: 68] [Impact Index Per Article: 9.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibroproliferative alterations of the microvasculature leading to fibrosis and loss of function of the skin and internal organs. Gastrointestinal manifestations of SSc are the most commonly encountered complications of the disease affecting nearly 90% of the SSc population. Among these complications, the esophagus and the anorectum are the most commonly affected. However, this devastating disorder does not spare any part of the gastrointestinal tract (GIT), and includes the oral cavity, esophagus, stomach, small and large bowels as well as the liver and pancreas. In this review, we present the current understanding of the pathophysiologic mechanisms of SSc including vasculopathy, endothelial to mesenchymal transformation as well as the autoimmune pathogenetic pathways. We also discuss the clinical presentation and diagnosis of each part of the GIT affected by SSc. Finally, we highlight the latest developments in the management of this disease, addressing the severe malnutrition that affects this vulnerable patient population and ways to assess and improve the nutritional status of the patients.
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Affiliation(s)
- Isabel M McFarlane
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Manjeet S Bhamra
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Alexandra Kreps
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Sadat Iqbal
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Firas Al-Ani
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Carla Saladini-Aponte
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Christon Grant
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Soberjot Singh
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Khalid Awwal
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Kristaq Koci
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Yair Saperstein
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Fray M Arroyo-Mercado
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Derek B Laskar
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
| | - Purna Atluri
- Division of Rheumatology and Gastroenterology, Department of Medicine and Pathology, Hospitals Kings County Hospital Brooklyn, State University of New York, USA
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Denaxas K, Ladas SD, Karamanolis GP. Evaluation and management of esophageal manifestations in systemic sclerosis. Ann Gastroenterol 2018; 31:165-170. [PMID: 29507463 PMCID: PMC5825946 DOI: 10.20524/aog.2018.0228] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/18/2017] [Accepted: 10/28/2018] [Indexed: 12/12/2022] Open
Abstract
Systemic sclerosis (SSc) is a multisystemic autoimmune connective tissue disorder; in the gastrointestinal tract, the esophagus is the most commonly affected organ. Symptoms of esophageal disease are due to gastroesophageal reflux disease (GERD) and esophageal motor dysfunction. Since the development of high-resolution manometry (HRM), this method has been preferred for the study of SSc patients with esophageal involvement. Using HRM, classic scleroderma esophagus, defined as absent or ineffective peristalsis of the distal esophagus in combination with a hypotensive lower esophageal sphincter, was found in as many as 55% of SSc patients. Endoscopy is the appropriate test for evaluating dysphagia and identifying evidence and possible complications of GERD. In the therapeutic area, treatment ranges from general supportive measures to the administration of drugs such as proton pump inhibitors and/or prokinetics. However, as many SSc patients do not respond to existing therapies, there is an urgent need for new therapeutic modalities. Buspirone, a 5-hydroxytryptamine 1A receptor agonist, could be a putative therapeutic option, as it was found to exert a significant beneficial effect in SSc patients with esophageal involvement. This review summarizes our knowledge concerning the evaluation and management of esophageal manifestations in SSc patients, including emerging therapeutic modalities.
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Affiliation(s)
- Konstantinos Denaxas
- Academic Department of Gastroenterology, National and Kapodistrian University of Athens, “Laikon” General Hospital, Athens, Greece
| | - Spyros D. Ladas
- Academic Department of Gastroenterology, National and Kapodistrian University of Athens, “Laikon” General Hospital, Athens, Greece
| | - George P. Karamanolis
- Academic Department of Gastroenterology, National and Kapodistrian University of Athens, “Laikon” General Hospital, Athens, Greece
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24
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25
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26
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Thompson JS, Langenfeld SJ, Hewlett A, Chiruvella A, Crawford C, Armijo P, Oleynikov D. Surgical treatment of gastrointestinal motility disorders. Curr Probl Surg 2016; 53:503-549. [PMID: 27765162 DOI: 10.1067/j.cpsurg.2016.08.006] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/20/2016] [Accepted: 08/22/2016] [Indexed: 12/11/2022]
Affiliation(s)
- Jon S Thompson
- Department of Surgery, University of Nebraska Medical Center, Omaha, NE.
| | - Sean J Langenfeld
- Department of Surgery, University of Nebraska Medical Center, Omaha, NE
| | - Alexander Hewlett
- Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE
| | | | | | | | - Dmitry Oleynikov
- Department of Surgery, University of Nebraska Medical Center, Omaha, NE
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27
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Tokman S, Hays SR, Leard LE, Bush EL, Kukreja J, Kleinhenz ME, Golden JA, Singer JP. Prolonged Barium-Impaction Ileus in Two Lung Transplant Recipients With Systemic Sclerosis: Case Report. Transplant Proc 2016; 47:2965-7. [PMID: 26707322 DOI: 10.1016/j.transproceed.2015.10.046] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/27/2015] [Accepted: 10/20/2015] [Indexed: 11/15/2022]
Abstract
Lung transplantation can be a life-saving measure for people with end-stage lung disease from systemic sclerosis. However, outcomes of lung transplantation may be compromised by gastrointestinal manifestations of systemic sclerosis, which can involve any part of the gastrointestinal tract. Esophageal and gastric disease can be managed by enteral feeding with the use of a gastrojejunal feeding tube. In this report, we describe the clinical courses of 2 lung transplant recipients with systemic sclerosis who experienced severe and prolonged barium-impaction ileus after insertion of a percutaneous gastrojejunal feeding tube.
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Affiliation(s)
- S Tokman
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of California, San Francisco, California; Advanced Lung Disease and Lung Transplant Program, Norton Thoracic Institute, St Joseph's Hospital and Medical Center, Phoenix, Arizona.
| | - S R Hays
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of California, San Francisco, California
| | - L E Leard
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of California, San Francisco, California
| | - E L Bush
- Division of Cardiothoracic Surgery, Department of Surgery, University of California, San Francisco, California
| | - J Kukreja
- Division of Cardiothoracic Surgery, Department of Surgery, University of California, San Francisco, California
| | - M E Kleinhenz
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of California, San Francisco, California
| | - J A Golden
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of California, San Francisco, California
| | - J P Singer
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of California, San Francisco, California
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28
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Kumar S, Singh J, Kedika R, Mendoza F, Jimenez SA, Blomain ES, DiMarino AJ, Cohen S, Rattan S. Role of muscarinic-3 receptor antibody in systemic sclerosis: correlation with disease duration and effects of IVIG. Am J Physiol Gastrointest Liver Physiol 2016; 310:G1052-60. [PMID: 27173508 PMCID: PMC4935481 DOI: 10.1152/ajpgi.00034.2016] [Citation(s) in RCA: 50] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/27/2016] [Accepted: 03/31/2016] [Indexed: 01/31/2023]
Abstract
Gastrointestinal dysmotility in systemic sclerosis (SSc) is associated with autoantibodies against muscarinic-3 receptor (M3-R). We investigated the temporal course of the site of action of these autoantibodies at the myenteric neurons (MN) vs. the smooth muscle (SM) M3-R in relation to disease duration, and determined the role of intravenous immunoglobulin (IVIG) in reversing these changes. Immunoglobulins purified from SSc patients (SScIgG) were used to assess their differential binding to MN and SM (from rat colon) employing immunohistochemistry (IHC). Effect of SScIgG on neural and direct muscle contraction was determined by cholinergic nerve stimulation and bethanechol-induced SM contraction. Effects of IVIG and its antigen-binding fragment F(ab')2 on SScIgG binding were studied by enzyme-linked immunosorbent assay (ELISA) of rat colonic longitudinal SM myenteric plexus (LSMMP) lysate and to second extracellular loop peptide of M3-R (M3-RL2). SScIgG from all patients demonstrated significantly higher binding to MN than to SM. With progression of SSc duration, binding at MN and SM increased in a linear fashion with a correlation coefficient of 0.696 and 0.726, respectively (P < 0.05). SScIgG-mediated attenuation of neural and direct SM contraction also increased with disease duration. ELISA analysis revealed that IVIG and F(ab')2 significantly reduced SScIgG binding to LSMMP lysate and M3-RL2. Dysmotility in SSc occurs sequentially, beginning with SScIgG-induced blockage of cholinergic neurotransmission (neuropathy), which progresses to inhibition of acetylcholine action at the SM cell (myopathy). IVIG reverses this cholinergic dysfunction at the neural and myogenic receptors by anti-idiotypic neutralization of SScIgG.
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Affiliation(s)
- Sumit Kumar
- 1Department of Medicine, Division of Gastroenterology and Hepatology, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, Pennsylvania;
| | - Jagmohan Singh
- 1Department of Medicine, Division of Gastroenterology and Hepatology, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, Pennsylvania;
| | - Ramalinga Kedika
- 1Department of Medicine, Division of Gastroenterology and Hepatology, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, Pennsylvania;
| | - Fabian Mendoza
- 2Division of Rheumatology, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, Pennsylvania;
| | - Sergio A. Jimenez
- 3Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Philadelphia, Pennsylvania; and
| | - Erik S. Blomain
- 4Department of Pharmacology and Experimental Therapeutics, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, Pennsylvania
| | - Anthony J. DiMarino
- 1Department of Medicine, Division of Gastroenterology and Hepatology, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, Pennsylvania;
| | - Sidney Cohen
- 1Department of Medicine, Division of Gastroenterology and Hepatology, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, Pennsylvania;
| | - Satish Rattan
- Department of Medicine, Division of Gastroenterology and Hepatology, Thomas Jefferson University, Sidney Kimmel Medical College, Philadelphia, Pennsylvania;
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29
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Brady SM, Shapiro L, Mousa SA. Current and future direction in the management of scleroderma. Arch Dermatol Res 2016; 308:461-71. [PMID: 27139430 DOI: 10.1007/s00403-016-1647-6] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2015] [Revised: 02/23/2016] [Accepted: 04/08/2016] [Indexed: 12/19/2022]
Abstract
Scleroderma is a heterogeneous disease with a complex etiology. As more information is gained about the underlying mechanisms and the improved classifications of scleroderma subtypes, treatments can be better personalized. Improving scleroderma patients' early diagnosis before end organ manifestations occur should improve clinical trial design and outcomes. Two recently FDA-approved antifibrotics for idiopathic pulmonary fibrosis may be effective treatments in patients with pulmonary fibrosis secondary to scleroderma after further investigation. The potential impact of Nanobiotechnology in improving the efficacy and safety of existing antifibrotics and immunomodulators might present an exciting new approach in the management of scleroderma.
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Affiliation(s)
- Sean M Brady
- The Pharmaceutical Research Institute, Albany College of Pharmacy and Health Sciences, 1 Discovery Drive (Room 238), Rensselaer, NY, 12144, USA
| | - Lee Shapiro
- Division of Rheumatology, Steffens Scleroderma Center, Albany Medical College, Albany, NY, USA
| | - Shaker A Mousa
- The Pharmaceutical Research Institute, Albany College of Pharmacy and Health Sciences, 1 Discovery Drive (Room 238), Rensselaer, NY, 12144, USA.
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30
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Becker MO, Riemekasten G. Risk factors for severity and manifestations in systemic sclerosis and prediction of disease course. Expert Rev Clin Immunol 2015; 12:115-35. [PMID: 26558747 DOI: 10.1586/1744666x.2016.1115717] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
Systemic sclerosis (SSc, or scleroderma) is a rheumatic disease with distinct features that encompass autoimmunity, vascular lesions (vasculopathy) and tissue fibrosis. The disease has a high morbidity and mortality compared with other rheumatic diseases. This review discusses risk factors and markers that predict the disease course and the occurrence of disease manifestations, with an emphasis on major organ involvement. In addition, risk factors will be described that are associated with mortality in SSc patients. The review addresses the impact of recent developments on screening, diagnosis and risk stratification as well as the need for further research where data are lacking.
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Affiliation(s)
- Mike O Becker
- a Department of Rheumatology and Clinical Immunology , University Hospital Charité Berlin , Berlin , Germany
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31
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Abstract
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by autoantibody production, small-vessel vasculopathy, and skin and other organ fibrosis. The disease is clinically heterogeneous with most patients having some degree of skin sclerosis with varying organ system involvement. Early disease can be difficult to diagnose, especially with minimal skin sclerosis and absence of anti-nuclear antibody (ANA) positivity; however, studies have demonstrated early diagnosis is important as early treatment could potentially lead to better outcomes. New classification criteria have recently been published that have higher sensitivity for detecting early disease thus allowing for a broader spectrum of patients to be represented in clinical trials. Treatment guidelines have been published based on a limited number of randomized-controlled clinical trials; however, there are ongoing phase II and III clinical trials with novel agents that are promising and will change the treatment landscape over the next decade.
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Affiliation(s)
- Courtney J McCray
- Division of Rheumatology and Clinical Immunogenetics, Department of Internal Medicine, University of Texas Health Science Center-Houston (UTHSC-H), 6431 Fannin St. MSB 5.278, Houston, TX, 77030, USA
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32
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Gastrointestinal Manifestations, Malnutrition, and Role of Enteral and Parenteral Nutrition in Patients With Scleroderma. J Clin Gastroenterol 2015; 49:559-64. [PMID: 25992813 DOI: 10.1097/mcg.0000000000000334] [Citation(s) in RCA: 38] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
Scleroderma (systemic sclerosis) is an autoimmune disease that can affect multiple organ systems. Gastrointestinal (GI) involvement is the most common organ system involved in scleroderma. Complications of GI involvement including gastroesophageal reflux disease, small intestinal bacterial overgrowth, and chronic intestinal pseudoobstruction secondary to extensive fibrosis may lead to nutritional deficiencies in these patients. Here, we discuss pathophysiology, progression of GI manifestations, and malnutrition secondary to scleroderma, and the use of enteral and parenteral nutrition to reverse severe nutritional deficiencies. Increased mortality in patients with concurrent malnutrition in systemic sclerosis, as well as the refractory nature of this malnutrition to pharmacologic therapies compels clinicians to provide novel and more invasive interventions in reversing these nutritional deficiencies. Enteral and parenteral nutrition have important implications for patients who are severely malnourished or have compromised GI function as they are relatively safe and have substantial retrospective evidence of success. Increased awareness of these therapeutic options is important when treating scleroderma-associated malnutrition.
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Abstract
The gastrointestinal tract, affecting more than 90% of patients, is the internal organ most frequently involved in systemic sclerosis. Any part of the gastrointestinal tract can be affected, from the mouth to the anus. Patients often experience reduced quality of life and impaired social life. Although only 8% have severe gastrointestinal involvement, mortality is high in those patients. Recent studies on the pathophysiology of the disease highlight new mechanisms to explain gastrointestinal dysmotility, but treatment remains symptomatic. This article reviews the pathophysiology of the gastrointestinal tract and discusses the investigation and management of the disease.
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Affiliation(s)
- Genevieve Gyger
- Division of Rheumatology, Jewish General Hospital, McGill University, Suite A725, 3755 Cote St Catherine Road, Montreal, Quebec H3T 1E2, Canada.
| | - Murray Baron
- Division of Rheumatology, Jewish General Hospital, McGill University, Suite A725, 3755 Cote St Catherine Road, Montreal, Quebec H3T 1E2, Canada
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