Eric Dein1, Pei-Lun Kuo2, Yun Soo Hong2, Laura K Hummers3, Christopher A Mecoli3, Zsuzsanna H McMahan4. 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.
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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