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Scleroderma & the Gut: New Frontiers in Diagnosis & Tips on Management

Samantha C. Shapiro, MD  |  Issue: June 2022  |  May 12, 2022

The modified Medsger GI severity score assessed physician-reported GI clinical severity.6 This score includes five categories of GI symptomatology:

  • 0=normal (no GI symptoms);
  • 1=requiring GERD medications or an abnormal bowel series;
  • 2=requiring high-dose GERD medications and/or having small bowel dilation on radiography;
  • 3=episodes of pseudo-obstruction or malabsorption syndrome; and
  • 4=severe GI dysmotility requiring either supplemental enteral nutrition or total parenteral nutrition.

The UCLA GIT 2.0 assessed patient-reported GI symptoms.7 This 34-question survey evaluates a variety of GI symptoms common in patients with SSc. Scores range from zero to 2.83, with higher scores indicating more severe symptomatology and worse health-related quality of life. 

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The majority of patients studied were female (85.9%) and white (78.6%), with limited cutaneous disease (70%). On cross-sectional analysis, the clinical and serologic characteristics of enrolled patients with SSc were largely comparable to other patients in the Johns Hopkins Scleroderma Center cohort, with certain exceptions. Example: Patients in this study were more commonly anticentromere antibody positive (45% vs. 27%; P<0.01), and more study patients had severe GI disease (Medsger GI severity score of 3: 14.9% vs. 5.5%; P<0.01). Researchers intentionally enriched the study with “patients who had more severe GI disease so as to learn about the impact of abnormal transit on less frequently observed, but more severe SSc GI complications.”

Analysis

Transit times and percent emptying were compared between patients with SSc and controls. As expected, patients with SSc had a significantly higher prevalence of abnormal function and delayed transit time in the esophagus, stomach and colon. Delayed small bowel transit was also more common among patients with SSc than controls, but the difference did not reach statistical significance.

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Next, McMahan et al. compared the prevalence of delayed transit in each area of the gut to each category of Medsger GI severity “to determine whether distinct GI transit abnormalities [are associated] with specific clinical SSc GI complications.” In patients with a Medsger score of 4 (total parenteral nutrition dependence), 100% (i.e., three of three patients) had small bowel abnormalities on whole gut transit scintigraphy. Among patients with a Medsger score of 3 (i.e., pseudo-obstruction and malabsorption), colonic transit was severely delayed.

Lastly, McMahan et al. compared UCLA GIT 2.0 questionnaire responses with abnormalities seen on whole gut transit scintigraphy to determine whether GI symptoms were associated with specific GI transit abnormalities.

Results

In this novel study, McMahan et al. demonstrated that SSc bowel disease affects GI transit, and this abnormal transit accounts, in part, for both bowel symptoms and severity of GI disease. Patients with pseudo-obstruction or malabsorption were more likely to have severe colonic transit delays. One-third of these patients had almost zero colonic emptying whatsoever after 72 hours. Although the number of patients was small (n=3), those dependent on total parenteral nutrition were more likely to have small bowel involvement of disease. Patients with more substantial upper GI symptoms were more likely to have dysmotility of the esophagus and/or stomach.

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Filed under:ConditionsResearch RheumSystemic Sclerosis Tagged with:Arthritis Care & Researchgutrefractory gastroesophageal reflux disease (GERD)Sclerodermasystemic sclerosis (SSc)

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