TY - JOUR
T1 - Influence of interstitial lung disease on outcome in systemic sclerosis
T2 - A population-based historical cohort study
AU - Bauer, Philippe R.
AU - Schiavo, Dante N.
AU - Osborn, Thomas G.
AU - Levin, David L.
AU - Sauver, Jennifer St
AU - Hanson, Andrew C.
AU - Schroeder, Darrell R.
AU - Ryu, Jay H.
N1 - Funding Information:
Author contributions: Dr Bauer is guarantor of the manuscript and takes responsibility for the integrity of the data and the accuracy of the data analysis. Dr Bauer: contributed to the concept and design of the study; collection, analysis, and interpretation of data; and review and final approval of the manuscript. Dr Schiavo: contributed to the collection, analysis, and interpretation of data and drafting, review, and final approval of the manuscript. Dr Osborn: contributed to the analysis of each case from a rheumatology standpoint and review and final approval of the manuscript. Dr Levin: contributed to the analysis of each case from a radiology standpoint and review and final approval of the manuscript. Dr St. Sauver : contributed to concept and design of the study, analysis and interpretation of data, and review and final approval of the manuscript. Mr Hanson: contributed to the statistical analysis and interpretation of data and review and final approval of the manuscript. Mr Schroeder: contributed to statistical analysis and interpretation of data and review and final approval of the manuscript. Dr Ryu: contributed to design of the study, analysis and interpretation of data, and review and final approval of the manuscript. Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr Bauer was the recipient of the 2011 Walter and Leonor Annenberg Career Development Award in Pulmonary Medicine, Mayo Foundation. Drs Schiavo, Osborn, Levin, St. Sauver, and Ryu and Messrs Hanson and Schroeder have reported that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article. Role of sponsors: Funding covered the cost for protected time for data retrieval, interpretation of data and drafting of the manuscript, and statistical analyses. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Other contributions: This work was performed at Mayo Clinic, Rochester, Minnesota.
PY - 2013/8
Y1 - 2013/8
N2 - Background: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and a major cause of SSc-related deaths. This study aimed to determine the infl uence of ILD on SSc in a population-based historical cohort study. The hypothesis was that patients with SSc who develop ILD have increased morbidity and mortality when compared with patients with SSc without ILD. Methods: Using the record linkage system of the Rochester Epidemiology Project in Olmsted County, Minnesota, this study identifi ed the incidence of SSc between 1980 and 2010 and point prevalence on December 31, 2010 and determined the progression of organ involvement and its infl uence on outcome. Results: During the 30-year interval, we identifi ed 64 incident cases of SSc: 57 women and seven men, median age 49.1 years (interquartile range [IQR], 39.8-67.6 years). There were 43 prevalent cases. ILD occurred in 19 cases, usually after the diagnosis of SSc (median, 2 years; IQR, 0-10 years), with only three cases occurring 6 to 24 months beforehand. Pulmonary arterial hypertension (PAH) was diagnosed in 14 cases, heart failure in 27 cases, and chronic kidney disease (CKD) in 21 cases. Seventeen patients died during the study period, with a median survival time after diagnosis of 22.9 years. ILD, PAH, and CKD were associated with an increased risk of death. Conclusions: The incidence of ILD associated with SSc was relatively low in this population-based cohort. ILD appeared to be a contributing factor to mortality. Other factors, including age, PAH, and CKD, were also associated with poor outcome.
AB - Background: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and a major cause of SSc-related deaths. This study aimed to determine the infl uence of ILD on SSc in a population-based historical cohort study. The hypothesis was that patients with SSc who develop ILD have increased morbidity and mortality when compared with patients with SSc without ILD. Methods: Using the record linkage system of the Rochester Epidemiology Project in Olmsted County, Minnesota, this study identifi ed the incidence of SSc between 1980 and 2010 and point prevalence on December 31, 2010 and determined the progression of organ involvement and its infl uence on outcome. Results: During the 30-year interval, we identifi ed 64 incident cases of SSc: 57 women and seven men, median age 49.1 years (interquartile range [IQR], 39.8-67.6 years). There were 43 prevalent cases. ILD occurred in 19 cases, usually after the diagnosis of SSc (median, 2 years; IQR, 0-10 years), with only three cases occurring 6 to 24 months beforehand. Pulmonary arterial hypertension (PAH) was diagnosed in 14 cases, heart failure in 27 cases, and chronic kidney disease (CKD) in 21 cases. Seventeen patients died during the study period, with a median survival time after diagnosis of 22.9 years. ILD, PAH, and CKD were associated with an increased risk of death. Conclusions: The incidence of ILD associated with SSc was relatively low in this population-based cohort. ILD appeared to be a contributing factor to mortality. Other factors, including age, PAH, and CKD, were also associated with poor outcome.
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U2 - 10.1378/chest.12-2768
DO - 10.1378/chest.12-2768
M3 - Article
C2 - 23450327
AN - SCOPUS:84881531904
SN - 0012-3692
VL - 144
SP - 571
EP - 577
JO - Chest
JF - Chest
IS - 2
ER -