Evaluation of risk factors for pseudo-obstruction in systemic sclerosis

Eric Dein, Pei Lun Kuo, Yun Soo Hong, Laura Hummers, Christopher Mecoli, Zsuzsanna McMahan

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalSeminars in Arthritis and Rheumatism
DOIs
StatePublished - Jan 1 2019

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Systemic Scleroderma
Autoantibodies
Opioid Analgesics
Phenotype
Muscular Diseases
DNA-Directed RNA Polymerases
Skin Diseases
Cross-Sectional Studies
Demography
Databases

ASJC Scopus subject areas

  • Rheumatology
  • Anesthesiology and Pain Medicine

Cite this

Evaluation of risk factors for pseudo-obstruction in systemic sclerosis. / Dein, Eric; Kuo, Pei Lun; Hong, Yun Soo; Hummers, Laura; Mecoli, Christopher; McMahan, Zsuzsanna.

In: Seminars in Arthritis and Rheumatism, 01.01.2019.

Research output: Contribution to journalArticle

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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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AB - 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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