Management of Gastrointestinal Involvement in Scleroderma

Nagaraja, Vivek; McMahan, Zsuzsanna H.; Getzug, Terri; Khanna, Dinesh

doi:10.1007/s40674-014-0005-0

Cited by 74 publications

(121 citation statements)

References 130 publications

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“…Although this study looked only at symptomatic endpoints rather than pH‐impedance testing, the findings suggest that there may be a role for these additional therapies in SSc patients. Promotility agents may improve LES tone and gastric emptying, thus improving reflux control, although the effects on esophageal contractility may wane with progressive smooth muscle atrophy and fibrosis …”

Section: Discussionmentioning

confidence: 99%

“…Up to 80% of patients with SSc have esophageal involvement . The most common esophageal disorder in SSc patients is gastro‐esophageal reflux disease (GERD) . Complications from esophageal reflux can be serious and include bleeding, stricture formation, Barrett's esophagus, and adenocarcinoma .…”

Section: Introductionmentioning

confidence: 99%

“…The most common esophageal disorder in SSc patients is gastro‐esophageal reflux disease (GERD) . Complications from esophageal reflux can be serious and include bleeding, stricture formation, Barrett's esophagus, and adenocarcinoma . In patients with SSc, uncontrolled acid reflux may contribute to the progression of interstitial lung disease .…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Abnormal esophageal acid exposure on high‐dose proton pump inhibitor therapy is common in systemic sclerosis patients

Stern

Carlson

Falmagne

et al. 2017

Neurogastroenterology Motil

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Abnormal esophageal acid exposure on high‐dose proton pump inhibitor therapy is common in systemic sclerosis patients

Stern

Carlson

Falmagne

et al. 2017

Neurogastroenterology Motil

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“…Therapeutic regimens often include (a) anti‐emetics for nausea and vomiting, (b) proton pump inhibitors and histamine‐2 receptor blockers for reflux symptoms, (c) antibiotics for small intestinal bacterial overgrowth (SIBO), (d) antidiarrheal agents or laxatives for bowel dysmotility and (e) prokinetics for dysmotility. Despite the wide array of medications listed above, their effect is very limited, especially when significant smooth muscle fibrosis has occurred …”

Section: What Is Known and Objectivementioning

confidence: 99%

Off‐label use of aprepitant for scleroderma‐associated nausea and vomiting: A case report

Jubran

Bolduc²,

Eng³

et al. 2019

J Clin Pharm Ther

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What is known and objective Scleroderma is a disease characterized by excessive deposition of collagen and extracellular matrix proteins in affected organ systems, which results in tissue fibrosis and organ dysfunction. It is estimated that 90% of patients with systemic sclerosis have gastrointestinal involvement, with approximately 50% being symptomatic. Clinical manifestations of gastrointestinal scleroderma manifestations relate to impaired motility and absorption and ultimately malnutrition. Treatments for these symptoms often fail and are limited. The objective of this case report is to highlight the potential use of a substance P antagonist in the treatment of scleroderma associated nausea. Case summary A 56‐year‐old woman presented with GI complications of her underlying systemic sclerosis. The majority of her stay was marked by severe nausea and vomiting, resistant to numerous therapies. Obstructive causes for her symptoms were ruled out with a gastroscopy. A motility study revealed completely absent peristalsis in her esophagus, likely due to complete fibrosis of smooth muscle fibres. For the first time, off‐label use of aprepitant 80 mg once daily was trialed with success in hospital. With this medication, she was able to maintain an adequate oral intake and ultimately achieve discharge from hospital. What is new and conclusion Despite the wide array of medications for gastrointestinal scleroderma, their effect is very limited. In this article, we report to the best of our knowledge the first successful use of the substance P antagonist aprepitant to treat refractory nausea and vomiting in a patient with gastrointestinal scleroderma. Not only does this medication carry promise in treating the GI symptoms of scleroderma, but it may also prove cost effective as compared to PEG insertions or TPN. This case study is congruent with recent evidence of the utility of aprepitant in other infiltrative disease conditions. This may spur interest randomized control trials for expanding the role of this medication.

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“…Gastrointestinal tract (GIT) involvement commonly affects patients with SSc, causing complications like malabsorption, weight loss and severe malnutrition [20]. In a recent study, it was reported that the Total GIT Score provides greater discrimination between mild, moderate, and severe self-rated GIT involvement than individual scales [21].…”

Section: Survival In Sscmentioning

confidence: 99%

Incidence and prevalence of Systemic Sclerosis (SSc) in Poland – differences between rural and urban regions

Kanecki

Goryński

Tarka

et al. 2017

Ann Agric Environ Med.

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Kanecki K, Goryński P,Tarka P, WierzbaW, Tyszko P. Incidence and prevalence of Systemic Sclerosis (SSc) in Poland -differences between rural and urban regions. Ann Agric Environ Med. 2017; 24(2): 240-244. doi: 10.5604/12321966.1233570 Abstract Introduction. Systemic sclerosis (SSc) is a rare and potentially severe connective tissue disease, characterized by skin fibrosis and involvement of internal organs. Because of its rarity and heterogeneous clinical presentation, reliable epidemiological studies on SSc have been particularly difficult to carry out. Objectives. The purpose of this study was to present SSc epidemiology among hospitalized patients in Poland. Materials and method. The analysis was based on population-based administrative data, taken from a Polish hospital morbidity study carried out by the National Institute of Public Health between January 2008 -December 2012. Results. Analyzed data covered 9,049 hospitalization records. The final sample comprised 3,653 patients with first-time hospitalizations for SSc. The average age of the sample was 53 years (SD 16.2; range 0-91 years); 84% of patients were female and 16% male. Based on hospitalization registers, the average SSc incidence was estimated to be 1.9/100,000 per year and peak age of incidence was 55 years. The point prevalence was estimated to be 9.4/100,000 at the end of 2012. SSc was more common in females, with F:M ratio ranging from 6.2:1-4.6:1 depending on the year. Analysis of hospitalization trends revealed that overall numbers of SSc hospitalizations increased, while first-time hospitalizations decreased between 2008-2012. Clusters of higher incidence were observed in more rural regions vs. urban regions. Conclusion. Estimated incidence of SSc in Poland was comparable to reported incidence in other European countries. Analysis of demographic factors and reports of clusters of higher incidence may suggest the existence of environmental risk factors for the development of SSc. These observations may require further research.

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Management of Gastrointestinal Involvement in Scleroderma

Cited by 74 publications

References 130 publications

Abnormal esophageal acid exposure on high‐dose proton pump inhibitor therapy is common in systemic sclerosis patients

Abnormal esophageal acid exposure on high‐dose proton pump inhibitor therapy is common in systemic sclerosis patients

Off‐label use of aprepitant for scleroderma‐associated nausea and vomiting: A case report

Incidence and prevalence of Systemic Sclerosis (SSc) in Poland – differences between rural and urban regions

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