Background/Purpose:

Although up to 90% of scleroderma (SSc) patients are affected by gastrointestinal (GI) dysmotility, clinical features of patients with severe lower gastrointestinal involvement are not well-defined. We sought to identify such features by studying a large cohort of SSc patients.

Methods:

We performed a retrospective analysis of patients seen during a 15-year period between 2002-2017 at The Johns Hopkins Scleroderma Center. All patients had data prospectively collected in a longitudinal database if they met 2013 ACR/EULAR criteria for systemic sclerosis, 1980 American College of Rheumatology (ACR) criteria or had at least three of five features of CREST syndrome. Lower gastrointestinal involvement was defined as ever having a Medsger’s GI severity score of a 3, which is characterized by malabsorption syndrome and/or recurrent episodes of pseudo-obstruction. Clinical and serologic characteristics of these patients were compared to those who received grades of 0, 1, or 2 throughout follow-up.

Results:

There were 193 patients with SSc who received a grade of 3 in GI severity during follow-up, and 3,577 patients who did not. In univariable analyses, lower GI involvement was significantly associated with older age at the time of severe bowel disease onset (58 vs. 53 years, p<0.001), diffuse cutaneous disease (47% vs. 37%; p=0.006), history of tendon friction rubs (24% vs. 16%; p=0.003), myopathy (31% vs. 17%; p <0.001), cancer (22% vs. 16%; p = 0.027), and death (44% vs. 34%; p=0.006). Such patients were also more likely to have ever had a lower FVC (66% vs. 70%, p =0.01) and DLCO (56 vs. 61%, p=0.02) and to have received opioids (44% vs. 27%; p<0.001), and prednisone (42% vs. 32%; p=0.005). A positive association with anti-U3RNP antibodies trended towards significance (7% vs. 4%; p=0.06). Autoantibodies to RNA polymerase 3 (RNApol3) were inversely associated with severe lower bowel involvement (11% vs. 18%; p = 0.02). In multivariable analyses (adjusted for significant variables from the univariable analysis) age (OR 1.03; 95%CI 1.02-1.05;), diffuse cutaneous disease (OR 1.76; 95%CI 1.16-2.67), myopathy (OR 1.56; 95%CI 1.06-2.30), and opioid use (OR 2.31; 95%CI 1.64-3.25) remained significantly associated with severe lower bowel disease in SSc. Anti-RNA pol3 antibodies were protective of this complication (OR 0.39; 95%CI 0.22-0.68).

Conclusion:

We report extra-intestinal clinical features of SSc patients with lower GI involvement in a large cohort. Our results demonstrate that older age, diffuse skin disease, and myopathy are associated with developing lower GI involvement. The prescription of opioids may be a modifiable risk factor for the development of severe lower GI involvement in patients with SSc.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-associated-with-severe-lower-bowel-involvement-in-systemic-sclerosis/