Clinical Assessment of Gastrointestinal Involvement in Patients with Systemic Sclerosis

Abstract

Systemic sclerosis (SSc) is an autoimmune connective tissue disease that negatively impacts the function of the skin and internal organs. The gastrointestinal (GI) tract is the most commonly affected internal organ in SSc, though GI complications may also arise indirectly when infections occur in the setting of immunosuppression or concurrent disease processes arise for which patients with SSc are found to be at higher risk. In this review, we provide a systematic approach for the clinical assessment of GI complications in SSc from the oropharynx to the anorectum to guide both general internists and rheumatologists caring for this complex patient population. It is organized so that each component of the luminal GI tract has its own specified section, beginning with a review of a clinical approach to diagnosis, followed by a more detailed discussion of the literature surrounding approaches for an objective GI evaluation. A focused discussion early in the manuscript addressing what is known about pathogenesis, and later about in the manuscript about the assessment for GI bleeding are also included.

Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis

“…The involvement of the GI tract is present in more than 90% of patients with SSc, however, the complaints are often non-specific and highly heterogeneous, making their diagnosis and management a challenge 7 12. This is worrisome as GI involvement is the leading cause of morbidity and third most common cause of mortality in SSc, being responsible for 3.5% of deaths, especially secondary to severe intestinal involvement, where mortality rates can reach 85% at 9 years 7 13–16. It is also associated with a significantly reduced quality of life, especially in the social functioning domain 17…”

“…Little is known regarding GI involvement pathophysiology, as it seems to move away from the mechanisms involved in other organ systems. Vasculopathy, autoimmunity, fibrosis and neuropathy have all been proposed to be involved in this, but contribution of diet, microbiota, comorbidities and drug iatrogenic reactions also play an important role 7 16…”

“…The small bowel is the second most frequently involved region of the GI tract (after oesophageal disease), affecting up to 88% of patients and manifesting as abdominal pain and distension, bloating and diarrhoea, as a consequence of dysmotility 16. Two of the most feared complications are the development of SIBO (43% of patients) and chronic intestinal pseudo-obstruction (3.9% of patients), especially in diffuse cutaneous SSc of more than 3 years duration 18 19.…”

Gastrointestinal involvement in systemic sclerosis: pathogenesis, assessment and treatment

“…oropharyngeal dysphagia, reflux, heartburn), lower oesophageal sphincter (LES) dysfunction [39], oesophageal dysmotility (distal dysphagia) and gastroparesis (e.g. early satiety, bloating, nausea, vomiting and unintentional weight loss) [1,40,41]. Symptoms may occur in isolation, or in combination, which can complicate both diagnosis and management.…”

“…Weight loss can occur, and although the differential diagnosis for weight loss in SSc is broad (Table 3), malabsorption should be considered in any patients with unintentional weight loss. Diagnostic tests include abdominal xrays, small intestinal manometry, scintigraphy, wireless motility capsules and computed tomography (CT)/MRI enterography [40] (Table 4). Small intestinal bacterial overgrowth (SIBO) [55] is estimated to occur in 30-62.5% of patients with SSc [56].…”