Sleep positional therapy for patients with systemic sclerosis and nighttime gastroesophageal reflux symptoms.

Loss of Peristaltic Reserve, Determined by Multiple Rapid Swallows, Is the Most Frequent Esophageal Motility Abnormality in Patients With Systemic Sclerosis.

AB0545 GASTROINTESTINAL INVOLVEMENT IN SYSTEMIC SCLEROSIS

BackgroundGastrointestinal (GI) tract is the second most affected organ in systemic sclerosis (SSc).ObjectivesThis study aims to analyze the frequency and severity of GI symptoms and their impact on health-related quality...

Fifty-five patients with progressive systemic sclerosis (PSS) were evaluated with esophageal manometry, 12-h pH-probe monitoring in esophagus, and registration of symptoms of gastroesophageal reflux (GER). Thirty-nine patients had symptoms suggestive of GER. The 12-h pH-monitoring showed pathologic GER in 30 patients. We found that 33% of the patients with symptoms did not have pathologic GER, and 25% of the patients without symptoms had pathologic GER. Characteristic changes of impaired motility in the esophagus were found in 46 patients. When combining esophageal manometry, reflux measurement, and symptoms of GER, we found a positive correlation in 60% of patients with reduced peristalsis in the lower two-thirds of the esophagus and GER. We therefore recommend sensitive esophageal pH-monitoring in all PSS patients to ensure treatment of patients with pathologic GER.

Abnormalities of esophageal and gastric emptying in progressive systemic sclerosis

The majority of systemic sclerosis (SSc) patients have gastrointestinal tract involvement, but therapies of prokinetic agents are usually unsatisfactory. Patients are often compromised by the use of steroid; therefore, a surgical indication including fundoplication has been controversial. There is no report that advanced SSc with severe gastroesophageal reflux disease (GERD) is successfully treated with acotiamide, which is the acetylcholinesterase (AChE) inhibitor designed for functional dyspepsia (FD). We report a 44-year-old woman of SSc with severe GERD successfully treated with acotiamide. She had received medical treatment in our hospital since 2003. She had been aware of the significant gastroesophageal reflux symptoms (e.g., heartburn, chest pain, and dysphagia) due to the development of esophageal hardening associated with SSc since 2014. As a result of upper gastrointestinal series, upper gastrointestinal endoscopy, and 24-h pH monitoring and frequency scale for the symptoms of the GERD (FSSG) scoring, she has been diagnosed with GERD associated with SSc. First of all, she started to take prokinetic agents Rikkunshito and mosapride and proton pump inhibitor; there was no change in reflux symptoms. So, we started to prescribe her the acotiamide.After oral administration started, reflux symptoms have been improved. Five months after oral administration, FSSG score, a questionnaire for evaluation of the symptoms of GERD, was improved. Since its introduction of acotiamide, the patient has kept free from symptoms for 6 months.

The gastrointestinal (GI) system frequently experiences complications in systemic sclerosis. Pathological changes in the GI system can be revealed from the early onset of the disease, such as esophageal hypomotility, to the late stage of the disease, where severe complications like intestinal pseudo-obstruction may occur. Systemic sclerosis can cause pathological changes throughout the entire gastrointestinal tract. Common symptoms include dysphagia, choking, heartburn, hoarseness, abdominal distension, diarrhea, constipation, and fecal incontinence. Oropharyngeal involvement in systemic sclerosis presents as weakened pharyngeal muscles, leading to oropharyngeal dysphagia, choking, food leakage, and aspiration, attributed to the dysfunction between the pharyngeal muscles and the upper esophageal sphincter due to muscle weakness. However, weak pharyngeal muscles may also be a clinical feature of the systemic sclerosis-polymyositis overlap syndrome. Esophageal involvement is frequently observed, with conditions such as esophageal hypomotility and lower esophageal sphincter incompetence, leading to early symptoms of dysphagia, gastroesophageal reflux, and potential complications such as esophagitis, esophageal stricture, Barrett’s esophagus, and aspiration pneumonia. Furthermore, gastric involvement includes gastric antral vascular ectasia, commonly known as “watermelon stomach,” which leads to gastrointestinal bleeding and chronic anemia. Intestinal manifestations comprise malabsorption, intestinal pseudo-obstruction, and bacterial overgrowth. Additionally, wide-mouthed diverticulosis and fecal incontinence are frequently found. Liver involvement includes primary biliary cirrhosis, most prevalent in limited cutaneous systemic sclerosis or certain overlap syndromes rather than diffuse cutaneous systemic sclerosis. The severity of these changes varies among patients, ranging from asymptomatic to severe conditions, which may result in malnutrition. Severe and chronic GI involvement significantly impacts daily life, making normal eating difficult, diminishing the quality of life, and potentially causing depression. Managing patients with systemic sclerosis who have GI involvement requires a multidisciplinary approach, involving collaboration among rheumatologists, gastroenterologists, nutritionists, surgeons, and possibly psychiatrists. This teamwork is essential to achieve successful patient care outcomes.

Lifestyle Factors and Risk for Symptomatic Gastroesophageal Reflux in Monozygotic Twins

Interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) is associated with increased morbidity and mortality. Gastroesophageal reflux (GER) is considered a contributing factor in the pathogenesis of ILD. To characterize GER (acid and nonacid) in patients with SSc with and without ILD. Patients with SSc underwent pulmonary high-resolution computer tomography (HRCT) scan and 24-hour impedance-pH monitoring off-proton pump inhibitor therapy. The presence of pulmonary fibrosis was assessed using validated HRCT-scores. Reflux monitoring parameters included number of acid and nonacid reflux episodes, proximal migration of the refluxate, and distal esophageal acid exposure. Unless otherwise specified, data are presented as median (25th-75th percentile). Forty consecutive patients with SSc (35 female; mean age, 53 yr; range, 24-71; 15 patients with diffuse and 25 with limited SSc) were investigated; 18 (45%) patients with SSc had pulmonary fibrosis (HRCT score >or= 7). Patients with SSc with ILD had higher (P < 0.01) esophageal acid exposure (10.3 [7.5-15] vs. 5.2 [1.5-11]), higher (P < 0.01) number of acid (41 [31-58] vs. 19 [10-23]) and nonacid (25 [20-35] vs. 17 [11-19]) reflux episodes, and higher (P < 0.01) number of reflux episodes reaching the proximal esophagus (42.5 [31-54] vs. 15 [8-22]) compared with patients with SSc with normal HRCT scores. Pulmonary fibrosis scores (HRCT score) correlated well with the number of reflux episodes in the distal (r(2) = 0.637) and proximal (r(2) = 0.644) esophagus. Patients with SSc with ILD have more severe reflux (i.e., more reflux episodes and more reflux reaching the proximal esophagus). Whether or not the development of ILD in patients with SSc can be prevented by reflux-reducing treatments needs to be investigated.

Background:Systemic Sclerosis (SSc) is an autoimmune disease characterized by vasculopathy and organ fibrosis leading to visceral damage. It is further classified as limited or diffuse SSc based on cutaneous involvement....

Background:Systemic sclerosis (SSc) is an autoimmune disease of unknown etiology, characterized by fibrosis in the skin, vascular walls, and certain organs such as the lungs, digestive tract, heart, or kidneys....

Systemic sclerosis (SSc) is a connective tissue disorder which frequently involves the esophagus, with severe gastroesophageal reflux (GER) and dysphagia as clinical consequences of esophageal dysmotility. The relationship between the severity and extent of esophageal acid exposure and the specific manometric disturbances has received little attention. Similarly, a paucity of manometric data exists regarding pharyngeal/upper esophageal sphincter (UES) function in SSc patients. We prospectively studied 36 SSc patients using computerized solid-state manometric and ambulatory dual-pH (upper and lower esophageal) monitoring, to define further the relationship between esophageal dysmotility and severity of GER in these patients. Patients were separated for analysis into two subgroups based on the absence (group 1, N = 25) or presence (group 2, N = 11) of distal esophageal peristalsis. SSc disease variant (diffuse vs. limited) and duration of illness were inaccurate predictors of the presence and severity of esophageal involvement. The mean lower esophageal sphincter (LES) pressure for the SSc patients (15.8 +/- 1.2 mm Hg, mean +/- SE) was significantly lower (p < 0.01) than that for a control group (26.0 +/- 2.1 mm Hg). There was no significant difference between the mean LES pressure for group 1 (15.0 +/- 1.6 mm Hg) and group 2 (17.5 +/- 1.6 mm Hg) patients. Although distal esophageal aperistalsis was noted in 70% of patients, normal proximal esophageal contraction pressures were documented in all cases. Mean UES pressure was significantly (p < 0.01) lower in group 1 (52.5 +/- 4.6 mm Hg) than in group 2 (80.5 +/- 10.6 mm Hg). The mean duration of UES relaxation and the mean time interval between the onset of UES relaxation and onset of pharyngeal contraction were significantly (p < 0.05) shorter for group 1 than group 2 patients. Pharyngeal pressures, peristalsis, and other aspects of pharyngeal/UES coordination were normal. Excessive distal esophageal acid exposure was often seen in patients in both subgroups, but it was significantly (p < 0.01) greater in group 1. Excessive proximal esophageal acid exposure was documented only in patients with absent distal peristalsis. Linear regression analysis revealed a poor correlation between the severity of esophageal acid exposure and the LES pressure. Thus, the severity and extent of GER in SSc is most closely related to the integrity of distal esophageal peristalsis.

The gastrointestinal tract (GIT) is the most common extracutaneous organ system damaged in systemic sclerosis (SSc) and is the presenting feature in 10% of patients. The esophagus as the portion of the GIT is the most commonly affected and there is an association of gastroesophageal reflux (GER) with SSc interstitial lung disease (ILD). Thus, an aggressive treatment for GER is recommended in all SSc patients with ILD; however, it is recognized that a long-term benefit to this treatment is needed to understand its impact. In this case report we discuss the presence of eosinophilic esophagitis (EoE) in two SSc patients and discuss the role for early EGD in SSc patients with moderate-severe GER symptoms for tissue study. Assessment of esophageal biopsy specimens for the presence of eosinophils and possibly ANA can help elucidate disease pathogenesis and direct therapy, as the presence of EoE in SSc has important management considerations, particularly with regards to dietary modification strategies.

Fibrosis and atrophy of esophageal smooth muscle cells cause gastro-esophageal reflux and dysphagia in most patients with systemic sclerosis (SSc). Recent studies indicate that distensibility of the esophagogastric junction (EGJ), assessed with the Functional Lumen Imaging Probe (FLIP) may be a more sensitive and accurate measure of sphincter function than manometry. We aim to describe and compare distension parameters of the EGJ in a well-characterized group of patients with SSc. Twelve patients with SSc reporting reflux or dysphagia (11 women, median age 53 [range 35-72], duration of disease: 1-20years) were investigated using distensibility testing of the EGJ. Patients were compared with 11 healthy volunteers (HV) (10 women, median age 53 [range 40-68]). The pressure and minimum diameter along the EGJ during ramp distension were used for distensibility analysis. Patients with SSc had significantly lower EGJ yield pressure (median: 4.0mmHg [Inter Quartile Range (IQR): 2.8-7.7]) than HV (median: 6.2mmHg [IQR: 9.4-26]) (P=.007). Likewise, the pressure-strain elastic modulus was lower in SSc patients (median 1.73 kPa [IQR: 1.16-2.15]) than in HV (median 2.41 kPa [IQR: 1.85-2.67]) (P=.03), indicating the reduced resistance to distension in SSc patient. Patients with SSc and symptoms of reflux and dysphagia have significantly reduced resistance to distension of the EGJ.

Proton pump inhibitor use and treatment satisfaction in Spanish patients with systemic sclerosis: Insights from a nationwide survey.