Evaluation of risk factors for pseudo-obstruction in systemic sclerosis

Semin Arthritis Rheum. 2019 Dec;49(3):405-410. doi: 10.1016/j.semarthrit.2019.05.005. Epub 2019 May 23.


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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Autoantibodies / immunology*
  • Biopsy
  • Cross-Sectional Studies
  • Electromyography
  • Female
  • Follow-Up Studies
  • Humans
  • Incidence
  • Intestinal Pseudo-Obstruction / diagnosis
  • Intestinal Pseudo-Obstruction / epidemiology
  • Intestinal Pseudo-Obstruction / etiology*
  • Magnetic Resonance Imaging
  • Male
  • Retrospective Studies
  • Risk Assessment / methods*
  • Risk Factors
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / diagnosis
  • Scleroderma, Systemic / immunology
  • United States / epidemiology


  • Autoantibodies