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Prediction of organ involvement and survival in systemic sclerosis patients in the first 5 years from diagnosis

van den Hombergh, Wieneke M.T. ; Knaapen-Hans, Hanneke K.A. ; van den Hoogen, Frank H.J. ; Carreira, Patricia ; Distler, Oliver ; Hesselstrand, Roger LU ; Hunzelmann, Nicolas ; Vettori, Serena ; Fransen, Jaap and Vonk, Madelon C. (2019) In Journal of Scleroderma and Related Disorders
Abstract

Background: Organ involvement often occurs in early systemic sclerosis and has been related to premature death. Identifying patients at diagnosis at risk of developing early organ involvement would be useful to optimize screening and management strategies. Objective: To develop prediction models for the 5-year development of interstitial lung disease, pulmonary arterial hypertension and death. Methods: A European multicentre inception cohort was created. For modelling, predefined clinical variables with known predictive value at diagnosis were used. Univariate and multivariate regression analysis were done to select baseline predictors and build the prediction models. The models were tested using the area under the receiver operating... (More)

Background: Organ involvement often occurs in early systemic sclerosis and has been related to premature death. Identifying patients at diagnosis at risk of developing early organ involvement would be useful to optimize screening and management strategies. Objective: To develop prediction models for the 5-year development of interstitial lung disease, pulmonary arterial hypertension and death. Methods: A European multicentre inception cohort was created. For modelling, predefined clinical variables with known predictive value at diagnosis were used. Univariate and multivariate regression analysis were done to select baseline predictors and build the prediction models. The models were tested using the area under the receiver operating characteristic curve comparing observed and expected frequencies. Results: Of 735 patients, 23% developed interstitial lung disease, 8% developed pulmonary arterial hypertension 12% died. The interstitial lung disease model included diffuse cutaneous systemic sclerosis (OR = 1.8), systemic sclerosis disease duration < 3 years (OR = 1.4), puffy fingers (OR = 1.6), and anti-topoisomerase-I-antibodies (OR = 1.8). The pulmonary arterial hypertension model included age > 65 years (OR = 3.2), forced vital capacity < 70% (OR = 2.5) and diffusing capacity of the lung for carbon monoxide < 55% (OR = 1.9). Death was predicted best by age > 65 years (OR = 4.1), male gender (OR = 1.9), no anti-centromere antibodies (OR = 0.5), proteinuria (OR = 1.9), forced vital capacity < 70% (OR = 1.8) and pulmonary arterial hypertension at diagnosis (OR = 10.1). The area under the receiver operating characteristic was 0.66 (95% CI 0.64–0.67), 0.66 (95% CI 0.64–0.68) and 0.70 (95% CI 0.69–0.72), respectively. Conclusion: We have shown that it is possible to predict interstitial lung disease, pulmonary arterial hypertension and death using established variables already available at the moment of systemic sclerosis diagnosis. Discriminatory performance of the models was suboptimal. Further research including new variables is necessary to improve performance.

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author
organization
publishing date
type
Contribution to journal
publication status
epub
subject
keywords
interstitial lung disease, organ involvement, pulmonary hypertension, survival, Systemic sclerosis
in
Journal of Scleroderma and Related Disorders
publisher
SAGE Publications
external identifiers
  • scopus:85073781691
ISSN
2397-1983
DOI
10.1177/2397198319869564
language
English
LU publication?
yes
id
2c542d70-4cf3-4e2a-a458-301d72e560b7
date added to LUP
2019-11-01 11:44:11
date last changed
2020-01-13 02:29:55
@article{2c542d70-4cf3-4e2a-a458-301d72e560b7,
  abstract     = {<p>Background: Organ involvement often occurs in early systemic sclerosis and has been related to premature death. Identifying patients at diagnosis at risk of developing early organ involvement would be useful to optimize screening and management strategies. Objective: To develop prediction models for the 5-year development of interstitial lung disease, pulmonary arterial hypertension and death. Methods: A European multicentre inception cohort was created. For modelling, predefined clinical variables with known predictive value at diagnosis were used. Univariate and multivariate regression analysis were done to select baseline predictors and build the prediction models. The models were tested using the area under the receiver operating characteristic curve comparing observed and expected frequencies. Results: Of 735 patients, 23% developed interstitial lung disease, 8% developed pulmonary arterial hypertension 12% died. The interstitial lung disease model included diffuse cutaneous systemic sclerosis (OR = 1.8), systemic sclerosis disease duration &lt; 3 years (OR = 1.4), puffy fingers (OR = 1.6), and anti-topoisomerase-I-antibodies (OR = 1.8). The pulmonary arterial hypertension model included age &gt; 65 years (OR = 3.2), forced vital capacity &lt; 70% (OR = 2.5) and diffusing capacity of the lung for carbon monoxide &lt; 55% (OR = 1.9). Death was predicted best by age &gt; 65 years (OR = 4.1), male gender (OR = 1.9), no anti-centromere antibodies (OR = 0.5), proteinuria (OR = 1.9), forced vital capacity &lt; 70% (OR = 1.8) and pulmonary arterial hypertension at diagnosis (OR = 10.1). The area under the receiver operating characteristic was 0.66 (95% CI 0.64–0.67), 0.66 (95% CI 0.64–0.68) and 0.70 (95% CI 0.69–0.72), respectively. Conclusion: We have shown that it is possible to predict interstitial lung disease, pulmonary arterial hypertension and death using established variables already available at the moment of systemic sclerosis diagnosis. Discriminatory performance of the models was suboptimal. Further research including new variables is necessary to improve performance.</p>},
  author       = {van den Hombergh, Wieneke M.T. and Knaapen-Hans, Hanneke K.A. and van den Hoogen, Frank H.J. and Carreira, Patricia and Distler, Oliver and Hesselstrand, Roger and Hunzelmann, Nicolas and Vettori, Serena and Fransen, Jaap and Vonk, Madelon C.},
  issn         = {2397-1983},
  language     = {eng},
  publisher    = {SAGE Publications},
  series       = {Journal of Scleroderma and Related Disorders},
  title        = {Prediction of organ involvement and survival in systemic sclerosis patients in the first 5 years from diagnosis},
  url          = {http://dx.doi.org/10.1177/2397198319869564},
  doi          = {10.1177/2397198319869564},
  year         = {2019},
}