Progression of Esophageal Dysmotility in Systemic Sclerosis

Vischio, John; Saeed, Fahd; Karimeddini, Mozafareddin K.; Mubashir, Asia; Feinn, Richard; Caldito, Gloria; Striegel, Kim; Rothfield, Naomi F.

doi:10.3899/jrheum.110923

Cited by 31 publications

(19 citation statements)

References 18 publications

(26 reference statements)

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“…Symptoms include dysphagia, odynophagia, heartburn and regurgitation but patients can also present with other symptoms related to oesophageal reflux such as chronic cough, hoarseness, pharyngitis, laryngospasm or asthma. Although oesophageal involvement is present in both limited and diffuse SSc, a recent study concluded that oesophageal dysmotility is more common in patients with diffuse SSc and is more likely to deteriorate over time compared to patients with limited SSc . Another study that assessed oesophageal motor function by high‐resolution manometry found that severe oesophageal dysmotility is associated with longer duration of SSc and with the presence of interstitial lung disease .…”

Section: Resultsmentioning

confidence: 99%

Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis

Kumar

Singh

Rattan

et al. 2017

Aliment Pharmacol Ther

108

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SUMMARY Background Gastrointestinal tract involvement is a common cause of debilitating symptoms in patients with systemic sclerosis. There are no disease modifying therapies for this condition and the treatment remains symptomatic, largely owing to the lack of a clear understanding of its pathogenesis. Aim To investigate novel aspects of the pathogenesis of gastrointestinal involvement in systemic sclerosis To summarize existing knowledge regarding the cardinal clinical gastrointestinal manifestations of systemic sclerosis and its pathogenesis, emphasizing recent investigations that may be valuable in identifying potentially novel therapeutic targets. Methods Electronic (Pubmed/Medline) and manual Google search Results The gastrointestinal tract is the most common internal organ involved in systemic sclerosis. Any part of the gastrointestinal tract from the mouth to the anus can be affected. There is substantial variability in clinical manifestations and disease course and symptoms are non-specific and overlapping for a particular anatomical site. Gastrointestinal involvement can occur in the in the absence of cutaneous disease. Up to 8% of systemic sclerosis patients develop severe gastrointestinal tract symptoms. This subset of patients display increased mortality with only 15% survival at 9 years. Dysmotiity of the gastrointestinal tract causes the majority of symptoms. Recent investigations have identified a novel mechanism in the pathogenesis of gastrointestinal tract dysmotility mediated by functional anti-muscarinic receptor autoantibodies. Conclusion Despite extensive investigation the pathogenesis of gastrointestinal involvement in systemic sclerosis remains elusive. Although treatment currently remains symptomatic, an improved understanding of novel pathogenic mechanisms may allow the development of potentially highly effective approaches including intravenous immunoglobulin and microRNA based therapeutic interventions.

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Section: Resultsmentioning

confidence: 99%

Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis

Kumar

Singh

Rattan

et al. 2017

Aliment Pharmacol Ther

108

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“…Akin to our finding of an association with more severe skin disease assessed by MRSS and more pronounced esophageal dysmotility (i.e., absent contractility), Vischio et al . found a correlation between MRSS and abnormal esophageal transit scintigraphy . Dermal skin thickness, which is caused by increased collagen deposition, is thought to correlate with more severe internal organ involvement, morbidity, and mortality .…”

Section: Discussionmentioning

confidence: 97%

“…Akin to our finding of an association with more severe skin disease assessed by MRSS and more pronounced esophageal dysmotility (i.e., absent contractility), Vischio et al found a correlation between MRSS and abnormal esophageal transit scintigraphy. 35 Dermal skin thickness, which is caused by increased collagen deposition, is thought to correlate with more severe internal organ involvement, morbidity, and mortality. 34,[36][37][38] A study utilizing endoscopic ultrasound identified thickened (compared with normal controls) esophageal walls, mainly in the submucosa and muscularis, in 14 patients with dcSSc and 11 lcSSc, which also correlated with the presence of dysphagia.…”

Section: Discussionmentioning

confidence: 99%

The association between systemic sclerosis disease manifestations and esophageal high‐resolution manometry parameters

Kimmel

Carlson

Hinchcliff

et al. 2016

Neurogastroenterology Motil

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Background/Aims We aimed to evaluate the associations between SSc-related systemic manifestations and esophageal function using high-resolution manometry (HRM). Methods Patients with SSc that had undergone HRM between 1/2004 and 9/2014 were identified and HRMs were analyzed according to the Chicago Classification. Clinical characteristics were identified via retrospective chart review and compared among motility diagnoses while adjusting for age, gender, race, and SSc-disease duration. Results 79 patients (85% female, ages 25–77) were included. Clinical characteristics were compared between patients with absent contractility (AC, n = 40), ineffective esophageal motility (IEM; n = 15), and normal motility (n = 19); the 5 remaining patients met criteria for other motility diagnoses. Groups differed in severity of skin involvement measured by the modified Rodnan skin score (0–51): AC (adjusted mean 12.6), IEM (4.4), normal (4.3), p = 0.043. Pulmonary function tests [percent predicted FVC and DLCO) were lower in AC (adjusted mean, FVC: 70.3, DLCO 51.1), than IEM (FVC: 92.0; DLCO: 76.9) and normal motility (FVC: 80.0; DLCO: 67.2), p-values 0.057 (FVC) and 0.007 (DLCO). Groups did not differ by SSc-disease duration, autoantibodies, or reported symptoms of dysphagia or reflux. Conclusions In patients with SSc, absent esophageal contractility on HRM was associated with increased skin disease severity and worse lung function. Obtaining HRM to identify SSc patients with more severe esophageal dysfunction could be considered to enable implementation of management strategies in patients potentially at risk for increased morbidity and mortality.

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“…This suggests the possibility of indirect assessment of interstitial lung disease severity, based on the degree of esophageal stagnation [17]. Finally, scintigraphy, being a simple and non-invasive study, seems to be an optimal diagnostic technique for routine monitoring of esophageal disease progression, as suggested by Vischio et al [18]. …”

Section: Significance Of Esophageal Transit Study In Systemic Sclerosismentioning

confidence: 99%

Esophageal transit scintigraphy in systemic sclerosis

2016

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Systemic sclerosis is a rare connective tissue disease, distinctive features of which are fibrosis and microangiopathy. The esophagus is one of the most commonly involved internal organs. Most patients experience dysphagia, difficulties in swallowing and gastro-esophageal reflux. However, in up to one third of cases, the initial onset of esophageal disease may be clinically silent. There are several diagnostic modalities available for assessing both morphological and functional abnormalities of the esophagus. If structural abnormalities are suspected, endoscopy is the method of choice. Functional evaluation is best achieved with manometry. Both endoscopy and manometry are invasive techniques, with low patient acceptance. Barium-contrast study is well tolerated, but qualitative assessment of functional abnormalities is imprecise. Esophageal scintigraphy is an easy, non-invasive, sensitive and specific diagnostic modality. It can detect esophageal dysfunction even in asymptomatic patients. In patients already diagnosed with systemic sclerosis, scintigraphy is useful in evaluating severity and progression of the disease.

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Progression of Esophageal Dysmotility in Systemic Sclerosis

Cited by 31 publications

References 18 publications

Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis

Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis

The association between systemic sclerosis disease manifestations and esophageal high‐resolution manometry parameters

Esophageal transit scintigraphy in systemic sclerosis

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