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Original Research: Diffuse Lung Disease |

Influence of Interstitial Lung Disease on Outcome in Systemic SclerosisSystemic Sclerosis and Interstitial Lung Disease: A Population-Based Historical Cohort Study

Philippe R. Bauer, MD, PhD, FCCP; Dante N. Schiavo, MD; Thomas G. Osborn, MD; David L. Levin, MD, PhD; Jennifer St. Sauver, PhD; Andrew C. Hanson, BS; Darrell R. Schroeder, MS; Jay H. Ryu, MD
Author and Funding Information

From Pulmonary and Critical Care Medicine (Drs Bauer, Schiavo, and Ryu), Rheumatology (Dr Osborn), Radiology-Diagnostic (Dr Levin), Health Sciences Research-Epidemiology (Dr St. Sauver), and Health Sciences Research-Biomedical Statistics and Informatics (Messrs Hanson and Schroeder), Mayo Clinic, Rochester, MN.

Correspondence to: Philippe R. Bauer, MD, PhD, FCCP, Pulmonary and Critical Care Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905; e-mail: bauer.philippe@mayo.edu


Funding/Support: This publication was made possible by Center for Translational Science Activities (CTSA) [Grant UL1 TR000135] from the National Center for Advancing Translational Sciences (NCATS), a component of the National Institutes of Health (NIH) and by Rochester Epidemiology Project [Grant R01 AG034676] from the National Institute on Aging. This work was also funded by the 2011 Walter and Leonor Annenberg Career Development Award in Pulmonary Medicine.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2013;144(2):571-577. doi:10.1378/chest.12-2768
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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 influence 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 identified the incidence of SSc between 1980 and 2010 and point prevalence on December 31, 2010 and determined the progression of organ involvement and its influence on outcome.

Results:  During the 30-year interval, we identified 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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