Gastrointestinal Involvement In Systemic Sclerosis Research Articles

The role of prokinetics in managing gastrointestinal involvement in systemic sclerosis: a systematic literature review.

Gastrointestinal involvement (GI) in systemic sclerosis (SSc) is frequent and heterogeneous, manifesting with different degrees of dysmotility. This systematic literature review aimed to summarize evidence on prokinetics for treating SSc-related GI dysmotility. Studies investigating the effects of prokinetic agents on GI function and/or GI symptoms in patients with SSc were systematically identified on PubMed and Embase. A qualitative data synthesis was conducted, given the (anticipated) wide heterogeneity in study designs, interventions, and outcomes. Twenty-one studies evaluating the effects of prokinetics in patients with SSc were included. Thirteen studies focused on GI motility using objective tests, eight assessed clinical responses, and six evaluated both. Cisapride (n = 5 studies), Metoclopramide (n = 7 studies), Octreotide (n = 4 studies), and Prucalopride (n = 1 study) were among the most studied prokinetics, with varying effects on different GI anatomical regions. While Metoclopramide consistently improved overall GI motility, other prokinetics provided selective benefits; Cisapride improved gastric emptying and colonic motility, but not esophageal motility, and Octreotide improved small bowel motility but delayed gastric emptying. Regarding symptomatic improvement, only prucalopride was evaluated using a validated patient questionnaire, showing improvement in both upper and lower GI symptoms. Prokinetic drugs may improve GI motility and symptoms in patients with SSc. There is an unmet need for future well-designed studies to refine patient stratification and optimize treatment outcomes.

Clinical characteristics and clinical risk model of the lower gastrointestinal involvement in systemic sclerosis.

Systemic sclerosis (SSc) is a systemic autoimmune disease that could involve multiple organs. The lower gastrointestinal involvement (LGIT) in systemic sclerosis (SSc-LGIT) is a serious manifestation and has a poor prognosis. This study aims to explore the related risk factors of SSc-LGIT that require hospitalisation and build a clinical risk model. SSc patients with LGIT admitted to Peking Union Medical College Hospital (PUMCH) were enrolled between December 2003 and December 2023. The controls were selected in SSc patients without LGIT in the same period after matching age and gender at a ratio of 1:3. Clinical data of both groups was collected to build the SSc-LGIT clinical prediction model by machine learning using R software. A total of 42 SSc patients with LGIT and 126 matched SSc patients without LGIT were enrolled. Compared to the control group, SSc-LGIT patients had lower level of body mass index (BMI), haemoglobin (HB), albumin (ALB). Cardiomyopathy and puffy finger were more common, but arthritis/arthralgia was less. Higher hs-CRP and a higher rate of anti-Ro-52 antibody positivity were found in these patients. A multivariate analysis revealed BMI, cardiomyopathy, HB, ALB, hs-CRP as independent related factors for SSc-LGIT, and a clinical risk model containing these five items was built. A clinical risk model of SSc-LGIT was established and it has demonstrated capability in predicting the risk of severe lower gastrointestinal involvement in systemic sclerosis.

Doppler ultrasound, a noninvasive tool for the study of mesenteric arterial flow in systemic sclerosis: a cross-sectional study of a patient cohort with review and meta-analysis of the literature.

Gastrointestinal involvement (GI) is a frequent and troublesome complication of systemic sclerosis (SSc), whose etiology is poorly understood, though it is hypothesized that autoimmunity and progressive vasculopathy may play a role. Vasculopathy is considered one of the main pathogenetic pathways responsible for many of the clinical manifestations of SSc, and, therefore, studying the principal splanchnic vessels (i.e., superior mesenteric artery-SMA and inferior mesenteric artery-IMA) with Doppler Ultrasound (DUS) may provide further insights into measuring the progression of vasculopathy, evaluating its possible association with SSc GI symptoms, and determining whether it plays a role in the development or severity of SSc GI disease. A cohort of SSc patients consecutively recruited underwent DUS examination, and associations with GI (UCLA-GIT 2.0 questionnaire) and extraintestinal SSc characteristics were evaluated. Semiquantitative DUS parameters (resistive index-RI and pulsatility index-PI), were applied for splanchnic vessel assessment in SSc patients and healthy subjects (HS). Moreover, a review and meta-analysis of the literature to understand which the values of the main semiquantitative DUS parameters (RI and PI) are both in SSc patients and HS has been conducted. Seventy-eight patients completed DUS examinations and clinical assessments. 30 (39%) were classified as diffuse cutaneous SSc (dcSSC), 35 (45%) as limited cutaneous SSc (lcSSc) and 13 (17%) as sine scleroderma. A significant difference was found both for SMA RI (p for trend = 0.032) and SMA PI (p for trend = 0.004) between patients with sine scleroderma, lcSSc and dcSSc, with lower values observed in the sine scleroderma and lcSSc groups. IMA RI and PI were significantly correlated with GI symptoms such as fecal incontinence (ῥ -0.33, p = 0.008 and ῥ -0.30, p = 0.021, respectively). By multivariate analysis, significant associations were confirmed between SMA RI and SMA PI and mRSS (β 0.248, p = 0.030 and β 2.995, p = 0.004, respectively) and with bosentan (β 0.400, p = 0.003 and β 3.508, p = 0.001, respectively), but not with anticentromere antibody (ACA). No significant differences were found between the weighted median values of SMA RI and SMA PI of SSc patients compared to those of HS that were derived from the meta-analysis of the literature (p = 0.72 and p = 0.64, respectively). This cross-sectional study confirms that the splanchnic vasculature of SSc patients can noninvasively been studied with DUS. Vascular splanchnic involvement correlates with the presence and/or severity of specific clinical features in SSc, including GI. Larger and prospective studies are needed to confirm these preliminary observations and to examine the role of DUS in SSc-risk stratification and GI progression and to obtain definitive data regarding both HS and SSc patients splanchnic DUS parameters.

Efficacy assessment of intravenous immunoglobulin for gastrointestinal involvement in systemic sclerosis using UCLA SCTC GIT: Case-based review.

To summarize the published evidence in the literature on the use of intravenous immunoglobulin in gastrointestinal tract involvement in systemic sclerosis patients and report the experience of our department. A systematic literature review was performed; and a literature search was conducted in MEDLINE and Embase until 1/5/2024, using the participants, intervention, comparator and outcomes framework. Only full-text articles involving systemic sclerosis adults, submitted to intravenous immunoglobulin (at least one administration) to treat primary gastrointestinal tract manifestations. The outcome was the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 score to evaluate response to treatment. Two reviewers performed the assessment of data extraction and synthesis, independently. Four papers (two case reports and two retrospective studies) out of 35 references were included. In addition, we added two systemic sclerosis patients from our department in this review. In 25 systemic sclerosis patients, with various gastrointestinal tract manifestations, the intravenous immunoglobulin therapy was found to improve digestive tract symptoms in SSc patients, as shown by the decrease in the scores of University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0. No adverse events were reported, except for one case of low-grade fever post-administration. The results from this systematic literature review based on case series suggest that intravenous immunoglobulin may improve gastrointestinal tract symptoms assessed by the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 scale, with only minor reported adverse events, suggestive of an acceptable safety profile. We believe that this systematic literature review will contribute to shed light on the efficacy and safety aspects of intravenous immunoglobulin treatment in the management of gastrointestinal tract symptoms; and multicenter randomized placebo-controlled trials are urgently needed to foster progress in this field.

Gastrointestinal Involvement in Systemic Sclerosis - Review of 250 Patients’ Clinical, Radiological and Serological Profile - A Retrospective Study

Background: Gastrointestinal manifestations are common in Systemic Sclerosis, but studies in the Indian population are sparse. Methods: This is a retrospective study of 100 patients who presented to the Department of Clinical Immunology and Rheumatology between 2014 to 2019. The charts and investigations were reviewed for clinical and serological profile, and the investigations done were recorded. Results: 100 patients diagnosed with systemic sclerosis (SSc) who fulfilled the ACR/EULAR 2013 criteria were included in the study-81 patients were classified as diffuse SSc, 15 as limited SSc and 4 as Sine Scleroderma. In total, 72 out of 100 patients reported reflux symptoms and 4 reported chronic diarrhoea. The imaging studies done for the patients and the findings are summarised in Table 1; the indications for endoscopy and the findings are summarised in Table 2. 2 patients were diagnosed with Chronic liver disease. 90 out of 100 patients were treated with a combination of proton pump inhibitors (PPIs) and prokinetics and 10 patients with PPIs alone. H Pylori eradication and antifungal therapy as per scopy findings were administered. 3 patients with chronic diarrhoea were empirically treated with Rifaximin for probable SIBO with symptomatic improvement. Conclusions: GI involvement is common in Scleroderma patients (93%- symptomatic and subclinical combined); however, the symptoms are under reported. Complications of GERD are less common in our popular, probably due to early initiation of PPIs and prokinetics. HRCT identified esophageal dilatation in 79% of the patients. Patients with refractory symptoms need to undergo scopies to rule out infections and malignancies. Further studies are required for better assessment and management of GI symptoms.

OA20 Pneumatosis intestinalis with spontaneous pneumoperitoneum in a patient with systemic sclerosis-myositis overlap syndrome: a case report and review of the literature

OA20 Pneumatosis intestinalis with spontaneous pneumoperitoneum in a patient with systemic sclerosis-myositis overlap syndrome: a case report and review of the literature

Gastrointestinal involvement in systemic sclerosis: pathogenesis, assessment and treatment.

The majority of patients with systemic sclerosis (SSc) will experience involvement of their gastrointestinal over the course of their disease. Despite the high prevalence of gastrointestinal involvement in SSc, the strategies pertaining to the assessment and treatment for this clinical dimension of SSc have historically been limited. However, the present review highlights recent research contributions that enhance our understanding of SSc-GI patient subsets and provides updates on pathogenic mechanisms of disease, assessment and symptom-directed management. In the past few years, several studies have identified risk factors for more severe gastrointestinal disease in SSc and have provided insight to optimize diagnosis and management of SSc-GI symptoms. This article also provides a review of currently available investigations and therapies for individual SSc-GI disease manifestations and reflects on actively evolving areas of research, including our understanding the role of the gut microbiome in SSc. Here, we provide important updates pertaining to the risk stratification, assessment, diagnosis and management of SSc patients with gastrointestinal symptoms. These findings provide opportunities to enhance patient care and highlight exciting opportunities for future research.

Sklerodermie

Gastrointestinal involvement in systemic sclerosis (SSc) is clinically determined by esophageal functional disorders with dysfunction of esophageal peristalsis and decrease of gastroesophageal barrier function with increased gastroesophageal reflux and its complications. Gastrointestinal involvement in SSc is relevant for the prognosis and is responsible for a significant number of deaths. The pathophysiology includes vascular alterations, fibrosis and immune alterations that most likely occur in sequential order leading to esophageal damage. Symptoms that correlate with esophageal dysfunction are dysphagia, reflux, belching, dyspepsia, pyrosis and retrosternal pain. Weight loss, nausea, vomiting, cough and hoarseness can be additional symptoms. The diagnostics include esophagogastroduodenoscopy, high-resolution esophageal manometry and combined 24 h pH impedance monitoring. Therapy comprises the treatment of the underlying disease and consistent guideline-conform treatment of reflux.

PELVIC FLOOR MUSCLE EXERCISE AND PHYSICAL THERAPY MAY IMPROVE SYSTEMIC SCLEROSIS-ASSOCIATED FECAL INCONTINENCE

Gastrointestinal involvement in systemic sclerosis (SSc) is observed in up to 90% of patients. Resolution of some of these gastrointestinal complications is challenging without the support of physical therapy and rehabilitation. One of these complications, SSc-associated fecal incontinence, which can be devastating for those affected, is seen in up to 39%. Studies focusing on fecal incontinence and its treatment are scarce. The hypothesis presented herein suggests that pelvic floor muscle exercise, biofeedback therapy, and neuromodulation methods might be effective and safe treatment strategies for patients affected by this debilitating complication.

P156 Understanding the associations and impact of severe gastrointestinal involvement in systemic sclerosis: a structured approach

Abstract Background Gastrointestinal (GI) involvement in systemic sclerosis (SSc) has a profound negative impact on patients’ quality of life. However, due to the diversity in manifestations of SSc-associated GI disease, this impact has proven difficult to quantify and to associate with other clinical and demographic features, to identify those most at risk of severe disease. Methods Between September and December 2018, consecutive SSc patients attending a specialist scleroderma clinic completed the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 (UCLA SCTC GIT 2.0) questionnaire. Total score and separate domain scores (reflux, distention/bloating, diarrhoea, soilage, constipation, social functioning and emotional wellbeing) were calculated. Domain scores range between 0 and 3, with higher scores indicating greater severity. Associations between mean scores and demographic or clinical characteristics were analysed using Wilcoxon rank-sum test. Results In total, 224 SSc patients fully completed the UCLA SCTC GIT 2.0. Eighty-three percent were female, mean age was 58.2 years and mean disease duration was 14.3 years. Diffuse cutaneous systemic sclerosis (dcSSc) was present in 37.1%, and 22.3% had an overlap syndrome. The most common autoantibodies were anti-centromere (ACA) in 38.0%, anti-topoisomerase I (ATA) in 20.1%, anti-RNA polymerase in 11.2% and anti-U3RNP in 4.5% of patients. For the cohort, the total GIT score was 0.62±0.57 (mean±SD), correlating strongly with visual analogue scale self-reported impact of GI disease (Pearson’s r = 0.72, p < 0.001). Distribution of total and domain scores is summarised in Table 1. We found no association between demographic or clinical characteristics and total GIT score, reflux, distention/bloating, diarrhoea and social functioning. Constipation was most common among ATA + (score 0.69, p = 0.080 v ATA-) and least common among ARA+ subjects (score 0.25, p = 0.014 v ARA-). Soilage was more common in limited cutaneous SSc (score 0.56 v 0.29 in dcSSc, p = 0.048) and ACA+ subjects (score 0.67 v 0.33 in ACA-, p = 0.008). ACA+ patients also had worse emotional wellbeing (score 0.70 v 0.52 in ACA-, p = 0.014). Emotional wellbeing was more severely affected in females (score 0.62 v 0.43 in males, p = 0.078). Conclusion We found that moderate to severe GI disease affects nearly half of all subjects with SSc, which significantly contributes to disease-related morbidity. Disclosures F. Ahmed None. S.I. Nihtyanova None. S. Chatzinikolaou None. V.H. Ong None. C.D. Murray None. C.P. Denton None.

Gastrointestinal involvement in systemic sclerosis: Effects on morbidity and mortality and new therapeutic approaches.

The majority of research studies in systemic sclerosis focus largely on addressing skin and cardiopulmonary manifestations. Fewer studies assess the pathogenesis and treatment of gastrointestinal tract involvement in systemic sclerosis, despite the fact that the majority of patients with systemic sclerosis have gastrointestinal manifestations and these manifestations are a leading cause of death in systemic sclerosis. The present review provides a comprehensive update on morbidity and mortality outcomes related to gastrointestinal involvement in systemic sclerosis. This review also describes conventional and emerging approaches to managing gastrointestinal symptoms in systemic sclerosis. Recent developments in systemic sclerosis-gastrointestinal research efforts have revealed promising treatment targets, including specific auto-antibodies and microbiota alterations. This review will conclude with an overview of future research directions that may improve our understanding of systemic sclerosis-gastrointestinal involvement and ultimately help to alleviate suffering from this devastating dimension of systemic sclerosis.

The role of high-resolution manometry in the assessment of upper gastrointestinal involvement in systemic sclerosis: a systematic review.

Systemic sclerosis (SSc) affects the upper gastrointestinal (GI) system in 90% of patients. High-resolution manometry (HRM) assesses esophageal dysmotility, but its role in diagnosis and follow-up remains unclear. The objectives of this systematic review were to investigate the role of HRM in the assessment of SSc-associated upper GI involvement and to evaluate the correlation between HRM abnormalities and clinical characteristics and the effects of therapeutic interventions on HRM findings. Fifteen articles were included. Most (11/15) studies were of very good or good quality. Most studies assessed correlations between esophageal symptoms and esophageal dysmotility. Two studies assessed the effectiveness of buspirone and reported HRM findings. Studies assessing upper GI symptoms using validated questionnaires, such as the University of California Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 or Gastrointestinal Symptoms Severity Index score, found an association between absent contractility on HRM and upper GI symptoms, but even asymptomatic patients often have esophageal body dysmotility on HRM. Esophageal dysmotility positively correlates with the presence of interstitial lung disease on high-resolution computed tomography and reduced diffusion capacity (< 0.8 of predicted value). Trials investigating the effect of buspirone demonstrate both increased lower esophageal sphincter resting pressure and reduced upper GI symptoms. Most studies report on limited patient numbers and retrospective data. Potential bias was minimized using quality appraisal. HRM findings correlate to upper GI symptoms when assessed by validated questionnaires and can detect response to therapy in buspirone trials. Esophageal body dysmotility on HRM positively correlates with the presence of interstitial lung disease. KEY POINTS: • Esophageal body dysmotility on HRM correlates with presence of ILD. • HRM findings seem to correspond to clinical symptom alleviation in interventional trials, but data are still limited. • At present HRM, a procedure with a high negative burden to the patient, offers little to no role in the therapeutic strategy.

Gastrointestinal involvement in systemic sclerosis: an update.

This review provides important updates in systemic sclerosis (SSc)-related gastrointestinal disease, specifically focusing on the most recent literature. 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. 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.

Upper gastrointestinal involvement in systemic sclerosis: Findings in a real-life setting.

Gastrointestinal tract involvement is the most common visceral affectation in systemic sclerosis (SSc), but the manifestations may vary in extension and severity. Endoscopic and histopathological gastroesophageal findings were investigated in patients with SSc. A total of 79 consecutive patients with definite SSc were enrolled in a cross sectional study. Clinical data were collected, upper gastrointestinal endoscopy and biopsies from gastric mucosa were performed in all cases. Fifty-seven (72.1%) out of 79 SSc patients had gastroesophageal symptoms. The most frequent were dysphagia, present in 33 (41.7%) and gastroesophageal reflux symptoms in 23 (29.1%) patients. Out of the 79 patients, 22 were asymptomatic, but in 16 esophageal and gastric mucosa changes were endoscopically detected. Reflux esophagitis was found in 39 (49.3%) patients. The presence of esophageal manifestations was not related to the disease duration or with its other variables. Signs of gastritis were endoscopically described in 47 (59.4%) and confirmed on histopathologic examinations in 45 patients. In 31 patients without any endoscopic changes, 18 (22.7%) showed signs of gastritis on histopathologic examination. No significant statistical differences were found between symptomatic and asymptomatic patients or between those with limited cutaneous SSc and those with diffuse cutaneous SSc in terms of clinical, endoscopic or histopathological findings, except the higher proportion of hiatal hernia in symptomatic patients. The results of this study might suggest that upper gastrointestinal endoscopy should be performed during the early stage of the disease and then periodically in patients diagnosed with SSc, even in the absence of typical symptoms.

Gastrointestinal involvement in systemic sclerosis: diagnostic and therapeutic aspects

Системна склеродермія є рідкісним автоімунним розладом, який асоціюється з високою захворюваністю та смертністю. Характеризується фіброзом численних органів, зокрема шкіри, опорно-рухового апарату, легень, нирок, серця та шлунково-кишкового тракту (ШКТ). Найчастіше страждає ШКТ (у 90 % випадків). Патогенетичні механізми, котрі беруть участь у цьому захворюванні, охоплюють ендотеліальне ушкод­ження, васкулопатію і вивільнення цитокінів, які ініціюють імунну відповідь, відкладення колагену і, зреш­тою, фіброз. До клінічних виявів захворювання належать втрата нормальної перистальтики, гіпотонія, ригідність стінок і м’язова атрофія, які призводять до парезу і дилатації органів ШКТ, надмірного росту бактерій у кишечнику, хронічної кишкової псевдообструкції, діареї, запору. Стравохід є найчастіше ураженою частиною ШКТ, що виявляється гастроезофагеальною рефлюксною хворобою, езофагітом, ризиком розвитку інтерстиціальних захворювань легень і стравоходу Барретта та потребує ретельного спостереження для раннього виявлення злоякісної трансформації. Варіанти ведення хворих на системну склеродермію з ураженням ШКТ далекі від ідеалу з огляду на короткострокову ефективність і наявність багатьох побічних ефектів. Застосовують інгібітори протонної помпи, прокінетики, антибіотики та ендоскопічні втручання. Необхідно провести додаткові дослідження для виявлення терапевтичних методів, які можуть стримати фіброзний процес на ранніх стадіях захворювання, запобігаючи ускладненням і постійному пошкодженню життєво важливих органів. Протиревматичні препарати, котрі модифікують захворювання, не мають ефекту при ураженні ШКТ.

E069 The efficacy of dietary intervention on gastrointestinal involvement in systemic sclerosis: a systematic literature review

E069 The efficacy of dietary intervention on gastrointestinal involvement in systemic sclerosis: a systematic literature review

Gastrointestinal involvement in systemic sclerosis: diagnosis and management.

This review provides important updates in systemic sclerosis (SSc)-related gastrointestinal disease, with a particular focus on the diagnosis and management of dysmotility. In the past 2 years, several studies were published that present interesting diagnostic insights into SSc and gastrointestinal dysmotility. Studies focusing on new therapies and the novel application of existing therapies, both in SSc and non-SSc-associated gastrointestinal dysmotility syndromes, demonstrate progress in the management of these challenging complications. SSc gastrointestinal disease is heterogeneous in its clinical presentation, which presents a challenge in diagnosis and management. Objective studies may help to identify patterns of gastrointestinal dysmotility and more specifically target therapy. A variety of drugs are now available or are under study in the management of gastrointestinal dysmotility, such as prucalopride, intravenous immunoglobulin, pyridostigmine, linaclotide, relamorelin, and others. These drugs may improve symptoms and quality of life in SSc gastrointestinal patients. Combination therapies are also under study. Electroacupuncture, dietary intervention (e.g. medical nutrition therapy, low FODmap diet), and medical cannibus may also play a role in alleviating patient symptoms; however, more data are needed to define the role of these interventions in SSc.

Gastrointestinal Involvement in Systemic Sclerosis: An Update.

Gastrointestinal Involvement in Systemic Sclerosis: An Update.

Progress of gastrointestinal involvement in systemic sclerosis

Systemic sclerosis is an autoimmune disease characterized by skin thickening and tightness. Digestive system is the most commonly damaged system secondary to skin, and as to unspecific clinical manifestations, this complication is often ignored in the early, the prognosis and quality of life of patients will be affected. This article overviews the recent research developments on pathology, clinical features, management of systemic sclerosis with digestive system involvement, to draw attention to early diagnosis and treatment of this condition for the improvement of the prognosis. Key words: Scleroderma, systemic; Digestive system; Disease attributes; Diagnosis; Therapeutics

Improved Gastrointestinal Involvement in Systemic Sclerosis after Immunoglobulin Treatment

전신경화증 환자는 위장관 침범을 하는 경우가 많으나 효과적인 치료법은 부족하다. 저자 등은 식도 내압 검사, 24시간 산도 검사 및 설문조사를 통해 광범위 전신경화증 환자의 위장관 침범을 확인하였고 면역글로불린을 투여하여 치료하였다. 이는 국내에서는 드문 증례이므로 문헌고찰과 함께 이를 보고하는 바이다.