“…In this study, a lower incidence of ILD was observed in the ANAnegative group, which agreed with data from Hamaguchi et al [9] based on 203 Japanese SSc patients. Despite being a multifactorial process, the etiology of GERD in SSc has been suggested to involve T lymphocyte-mediated activation of myo broblasts through cytokines and growth factors, resulting in excessive collagen production, which causes structural damage and brosis of normal esophageal tissues and also leads to dysmotility [20]. Our study showed that ANA-negative SSc patients were less likely to develop GERD.…”
Objective: The presence of circulating antinuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). A variety of ANAs are associated with unique sets of disease manifestations and are widely used in clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvement and prognosis in SSc. This study aimed to investigate the clinical features of SSc patients negative for ANAs in a Chinese Rheumatism Data Center (CRDC) multicenter cohort in China.Methods: Based on the CRDC database, patients were prospectively recruited between April 2008 and June 2019 from 154 clinical centers nationwide, and all cases fulfilled the 2013 ACR/EULAR classification criteria for systemic sclerosis. Results for antinuclear antibodies were intensively collected. Demographic, clinical, and laboratory data were compared between ANA-positive SSc patients and those negative for ANAs.Results: Antinuclear antibodies were detected in 2129 of 2809 patients enrolled in the study; 4.2% patients were negative. There was a significant difference between patients negative and positive for ANAs based on sex (29/60 vs 294/1746, p<0.001). The presence of Raynaud’s phenomenon was less common (71.8% vs 91.8%, p<0.001) in the ANA-negative patients. In addition, the incidence of certain critical organ involvement, including gastroesophageal reflux (5.6% vs 18.5%, p=0.002), interstitial lung disease (65.2% vs 77.9%, p=0.015) and pulmonary arterial hypertension (11.5% vs 29.0%, p=0.006), was significantly lower in ANA-negative patients than in ANA-positive patients. The proportion of abnormal ESR (32.4% vs 47.6%, p=0.013) and IgG elevation (14.3% vs 37.0%, p=0.003), an indicator of disease activity, was significantly lower in ANA-negative patients than in ANA-positive patients.Conclusion: Antinuclear antibodies are strongly associated with the clinical manifestations of systemic sclerosis, with ANA-negative SSc patients tending to exhibit relatively milder disease.
“…In this study, a lower incidence of ILD was observed in the ANAnegative group, which agreed with data from Hamaguchi et al [9] based on 203 Japanese SSc patients. Despite being a multifactorial process, the etiology of GERD in SSc has been suggested to involve T lymphocyte-mediated activation of myo broblasts through cytokines and growth factors, resulting in excessive collagen production, which causes structural damage and brosis of normal esophageal tissues and also leads to dysmotility [20]. Our study showed that ANA-negative SSc patients were less likely to develop GERD.…”
Objective: The presence of circulating antinuclear antibodies (ANAs) is a hallmark of immune dysregulation in patients with systemic sclerosis (SSc). A variety of ANAs are associated with unique sets of disease manifestations and are widely used in clinical practice for diagnosis, clinical subgrouping, and prediction of future organ involvement and prognosis in SSc. This study aimed to investigate the clinical features of SSc patients negative for ANAs in a Chinese Rheumatism Data Center (CRDC) multicenter cohort in China.Methods: Based on the CRDC database, patients were prospectively recruited between April 2008 and June 2019 from 154 clinical centers nationwide, and all cases fulfilled the 2013 ACR/EULAR classification criteria for systemic sclerosis. Results for antinuclear antibodies were intensively collected. Demographic, clinical, and laboratory data were compared between ANA-positive SSc patients and those negative for ANAs.Results: Antinuclear antibodies were detected in 2129 of 2809 patients enrolled in the study; 4.2% patients were negative. There was a significant difference between patients negative and positive for ANAs based on sex (29/60 vs 294/1746, p<0.001). The presence of Raynaud’s phenomenon was less common (71.8% vs 91.8%, p<0.001) in the ANA-negative patients. In addition, the incidence of certain critical organ involvement, including gastroesophageal reflux (5.6% vs 18.5%, p=0.002), interstitial lung disease (65.2% vs 77.9%, p=0.015) and pulmonary arterial hypertension (11.5% vs 29.0%, p=0.006), was significantly lower in ANA-negative patients than in ANA-positive patients. The proportion of abnormal ESR (32.4% vs 47.6%, p=0.013) and IgG elevation (14.3% vs 37.0%, p=0.003), an indicator of disease activity, was significantly lower in ANA-negative patients than in ANA-positive patients.Conclusion: Antinuclear antibodies are strongly associated with the clinical manifestations of systemic sclerosis, with ANA-negative SSc patients tending to exhibit relatively milder disease.
“…Patients in whom GERD has not been diagnosed or controlled may have serious complications, such as esophageal stricture, BE, esophageal adenocarcinoma, ILD, and pulmonary fibrosis[ 67 ]. Combined with the pulmonary manifestations typical of SSc, these latter complications predispose patients to end-stage lung disease and, ultimately, lung transplantation in refractory cases[ 68 ].…”
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.
“…Esophageal manometry and 24-h pH monitoring are commonly used in assessing esophageal peristalsis [24]. The typical manifestations of esophageal dyskinesia caused by SSc in esophageal manometry are the absence of contractility or weak peristalsis in the lower third of the esophagus and relaxation of the esophagogastric junction (EGJ) [38][39][40]. About 60-80% of patients with SSc have abnormal reflux, and 50-90% have abnormal pressure [23].…”
Section: Traditional Manometric Monitoring and Characteristicsmentioning
confidence: 99%
“…Most patients’ upper gastrointestinal endoscopic findings show esophagitis [ 36 ] without clear specific manifestations [ 4 , 17 , 23 , 37 ]. Early biopsy of the lower end of the esophagus commonly shows squamous cell gap widening, and late biopsy shows pathological manifestations related to its complications [ 38 ]. Endoscopic ultrasonography manifests with hyperechoic lamina propria and muscularis mucosa, suggesting the occurrence of fibrosis [ 2 ].…”
Section: Clinical Examination and Diagnosismentioning
Esophageal motility disorders are prevalent in 90% of patients with systemic sclerosis [scleroderma (SSc)], with an increased mortality rate in patients with severe esophageal involvement. Esophageal smooth muscle damage caused by ischemia, nerve damage, and inflammatory factors may be responsible for discomfort and various complications in these patients. The clinical manifestations are diverse. Most hospitals still use traditional esophageal manometry and 24-h pH monitoring to diagnose esophageal function in patients with SSc. The aim of this review article is to provide an overview of SSc-related esophageal motility disorders and related research progress, including the pathogenesis and clinical features of these disorders and the progress made in endoscopic diagnosis. We also discuss the possible pathogenesis and potential therapeutic targets.
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