@article{64abc5b8e6dd4c89bc59bc69de0dd952,
title = "A practical approach to the evaluation and management of gastrointestinal symptoms in patients with systemic sclerosis",
abstract = "The gastrointestinal (GI) tract is the most commonly affected internal organ system in systemic sclerosis (SSc). SSc may lead to impaired function in any region of the GI tract, from the esophagus to the anorectum, which causes significant morbidity as well as mortality in patient subsets. Given the low prevalence of SSc in the community, many rheumatologists may not have a systematic framework for diagnosing or treating the GI complaints in this disease. These practice recommendations aim to summarize and consolidate the current guidelines from the fields of gastroenterology and rheumatology and establish a symptom-based framework for diagnosis and management based on available evidence in the literature. Subject areas that are in need of additional research are also identified.",
keywords = "Gastrointestinal, Management, Scleroderma, Systemic sclerosis",
author = "Timothy Kaniecki and Tsion Abdi and McMahan, {Zsuzsanna H.}",
note = "Funding Information: If a Barium swallow study suggests dysphagia originating at the level of the oropharynx, overlap syndromes should be considered. The presence of sicca symptoms can be confirmed by a thorough history and physical exam, taking particular care to note any reported xerostomia (dry mouth), oral sores, or glossodynia that may contribute to dysphagia [17]. Salivary gland biopsies and Sjogren's Syndrome antibodies do not correlate with sicca symptom severity in patients with SSc, and thus are likely unnecessary as they would not affect management [18]. A comprehensive review of the diagnosis and treatment options of Sjogren's Syndrome and/or sicca was recently published here [17]. Elevations in serum creatinine kinase (CK), aldolase, or antibodies associated with autoimmune myositis or myasthenia gravis (e.g., anti-acetylcholine receptor or muscle-specific tyrosine kinase [MUSK] antibodies) would provide support for an underlying myopathy as a potential contributor to pharyngeal muscle weakness [14,19]. Although the identification of myositis or myasthenia gravis is important in the determination of optimal treatment strategies for oropharyngeal dysfunction, the nuances of their treatment are outside the scope of this review but may be found here [20,21]. Practice points for oropharyngeal dysphagia and/or globus sensation can be found in Table 3. The AGA guidelines for the management of GERD [24] outline that daily standard-dose proton pump inhibitors (PPIs) (see Table 4) should be considered as first-line therapy based on strong support from clinical trial data. PPIs are beneficial both for symptom management and for treating complications such as esophageal ulcers and strictures [7]. This recommendation is supported by a Cochrane review [26] that concluded that PPIs are superior to histamine-2 receptor antagonists (H2RAs), which in turn are superior to placebo in the treatment of symptomatic reflux and healing of esophagitis. No significant difference was found in the efficacy among different PPIs (esomeprazole, lansoprazole, omeprazole, pantoprazole, and rabeprazole) by meta-analysis. In patients who initially responded to therapy, this symptomatic benefit extended to the 6- and 12-month follow- up periods. While the only small, randomized control trial [27] for PPIs in SSc did not see benefit at 12-month follow up, this study only investigated lansoprazole and did not assess for outcomes related to esophageal healing. Acknowledging the limitations of this small study, chronic PPI therapy in SSc is guided by abundant data from the GI literature, with both the EULAR and the UKSSG guidelines in agreement with the AGA's recommendation [7,8].While limited data exists for the efficacy of twice daily dosing of PPIs, the AGA guidelines [24] state that gastroenterologist expert opinion supports this increased dosing for patients with breakthrough symptoms. The guidelines propose H2RAs as an alternative acid suppressant when patients experience significant adverse effects from PPIs [24]. Expert consensus across gastroenterologists and rheumatologists (when dealing with reflux in SSc) is that all medications should be titrated to optimize symptom control, while utilizing the lowest possible therapeutic medication dose [8,24]. Any patients who continue to experience symptoms despite maximal acid-suppression therapy should be considered “treatment failures” and should undergo additional diagnostic evaluation. Publisher Copyright: {\textcopyright} 2021 Elsevier Ltd",
year = "2021",
month = sep,
doi = "10.1016/j.berh.2021.101666",
language = "English (US)",
volume = "35",
journal = "Best Practice and Research: Clinical Rheumatology",
issn = "1521-6942",
publisher = "Bailliere Tindall Ltd",
number = "3",
}