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Semin Arthritis Rheum. 2019 May 23. pii: S0049-0172(19)30038-1. doi: 10.1016/j.semarthrit.2019.05.005. [Epub ahead of print]

Evaluation of risk factors for pseudo-obstruction in systemic sclerosis.

Author information

1
Johns Hopkins University School of Medicine, United States.
2
Johns Hopkins Bloomberg School of Public Health, United States.
3
Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Suite 5200, Mason F. Lord Building, Center Tower, Baltimore, MD 21224, United States.
4
Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Suite 5200, Mason F. Lord Building, Center Tower, Baltimore, MD 21224, United States. Electronic address: zmcmaha1@jhmi.edu.

Abstract

OBJECTIVE:

Although up to 90% of systemic sclerosis (SSc) patients are affected by gastrointestinal (GI) dysmotility, the clinical phenotype of patients with pseudo-obstruction is not well-defined. We sought to identify this phenotype by studying a large cohort of SSc patients with and without pseudo-obstruction.

METHODS:

We performed a retrospective analysis of patients seen at the Johns Hopkins Scleroderma Center between February 2003 and September 2017. All SSc patients had clinical data prospectively collected in a longitudinal database. Cross-sectional analyses were performed comparing autoantibody status and clinical and demographic features of patients with and without pseudo-obstruction. Cox proportional hazards regression was used to identify risk factors for pseudo-obstruction.

RESULTS:

175 patients with SSc had a history of pseudo-obstruction, and 2,637 SSc patients did not. After adjusting for significant variables from the univariate analysis and potential confounders, the Cox proportional hazards multivariable analysis demonstrated that older age (HR 1.02; 95%CI 1.00-1.04), male sex (HR 1.75; 95%CI 1.42-2.43), diffuse cutaneous disease (HR 2.52; 95%CI 1.59-3.99), myopathy (HR 1.83, 95%CI 1.09-3.08), and opioid use (HR 2.38; 95%CI 1.50-3.78) were predictive of pseudo-obstruction. Autoantibodies to RNA polymerase-3 were negatively associated with pseudo-obstruction (HR 0.34; 95%CI 0.17-0.66).

CONCLUSION:

We identified clinical features associated with pseudo-obstruction in a large US SSc cohort. This study identifies characteristics of patients with SSc who are at a higher risk of developing pseudo-obstruction and suggests that opioids may be a modifiable risk factor. These clinical features may allow for earlier diagnostic evaluation and/or therapeutic intervention for patients at risk for pseudo-obstruction.

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