Influence of interstitial lung disease on outcome in systemic sclerosis: A population-based historical cohort study

Philippe R. Bauer, Dante N. Schiavo, Thomas Osborn, David L. Levin, Jennifer St. Sauver, Andrew C. Hanson, Darrell R. Schroeder, Jay H Ryu

Research output: Contribution to journalArticle

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Abstract

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.

Original languageEnglish (US)
Pages (from-to)571-577
Number of pages7
JournalChest
Volume144
Issue number2
DOIs
StatePublished - Aug 2013

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Systemic Scleroderma
Interstitial Lung Diseases
Cohort Studies
Population
Chronic Renal Insufficiency
Pulmonary Hypertension
Mortality
Age Factors
Incidence
Epidemiology
Heart Failure
Morbidity
Survival

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Influence of interstitial lung disease on outcome in systemic sclerosis : A population-based historical cohort study. / Bauer, Philippe R.; Schiavo, Dante N.; Osborn, Thomas; Levin, David L.; St. Sauver, Jennifer; Hanson, Andrew C.; Schroeder, Darrell R.; Ryu, Jay H.

In: Chest, Vol. 144, No. 2, 08.2013, p. 571-577.

Research output: Contribution to journalArticle

Bauer, Philippe R. ; Schiavo, Dante N. ; Osborn, Thomas ; Levin, David L. ; St. Sauver, Jennifer ; Hanson, Andrew C. ; Schroeder, Darrell R. ; Ryu, Jay H. / Influence of interstitial lung disease on outcome in systemic sclerosis : A population-based historical cohort study. In: Chest. 2013 ; Vol. 144, No. 2. pp. 571-577.
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abstract = "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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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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