Universität zu Köln

van den Hombergh, Wieneke M. T., Knaapen-Hans, Hanneke K. A., van den Hoogen, Frank H. J., Carreira, Patricia, Distler, Oliver ORCID: 0000-0002-0546-8310, Hesselstrand, Roger, Hunzelmann, Nicolas, Vettori, Serena, Fransen, Jaap and Vonk, Madelon C. (2020). Prediction of organ involvement and survival in systemic sclerosis patients in the first 5years from diagnosis. J. Scleroderma Relat. Disord., 5 (1). S. 57 - 66. LONDON: SAGE PUBLICATIONS LTD. ISSN 2397-1991

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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 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<3years (OR = 1.4), puffy fingers (OR = 1.6), and anti-topoisomerase-I-antibodies (OR = 1.8). The pulmonary arterial hypertension model included age>65years (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>65years (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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code van den Hombergh, Wieneke M. T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Knaapen-Hans, Hanneke K. A. UNSPECIFIED UNSPECIFIED UNSPECIFIED van den Hoogen, Frank H. J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Carreira, Patricia UNSPECIFIED UNSPECIFIED UNSPECIFIED Distler, Oliver UNSPECIFIED orcid.org/0000-0002-0546-8310 UNSPECIFIED Hesselstrand, Roger UNSPECIFIED UNSPECIFIED UNSPECIFIED Hunzelmann, Nicolas UNSPECIFIED UNSPECIFIED UNSPECIFIED Vettori, Serena UNSPECIFIED UNSPECIFIED UNSPECIFIED Fransen, Jaap UNSPECIFIED UNSPECIFIED UNSPECIFIED Vonk, Madelon C. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-141648
DOI:	10.1177/2397198319869564
Journal or Publication Title:	J. Scleroderma Relat. Disord.
Volume:	5
Number:	1
Page Range:	S. 57 - 66
Date:	2020
Publisher:	SAGE PUBLICATIONS LTD
Place of Publication:	LONDON
ISSN:	2397-1991
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language PULMONARY ARTERIAL-HYPERTENSION; EULAR SCLERODERMA TRIALS; RISK-FACTORS; DISEASE; GUIDELINES; MANAGEMENT; MORTALITY; MODELS; DEATH Multiple languages Rheumatology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/14164