Beyond screening for pulmonary arterial hypertension: The DETECT score is a potential promising prediction tool for all-cause mortality in systemic sclerosis: Analysis from the EUSTAR database

Florian Käs; Muriel Elhai; Mike O Becker; Rucsandra Dobrota; Carina Mihai; Gesa Sauer; Lorenzo Tofani; Radim Bečvář; Simona Rednic; Patricia E Carreira; Gábor Kumánovics; Paolo Airò; Ulf Mueller-Ladner; Francesco Del Galdo; Ana-Maria Ramazan; Mickaël Martin; Carmen-Pilar Simeón-Aznar; Magda Parvu; Nicoletta Del Papa; Anna-Maria Hoffmann-Vold; Oliver Distler; Cosimo Bruni; Silvia Bellando-Randone; Ulrich Andreas Walker; Florenzo Iannone; Yannick Allanore; Michele Iudici; Elisabetta Zanatta; Gianluca Moroncini; Mislav Radic; Nico Hunzelmann; Luca Idolazzi; Joerg Henes; Bojana Stamenkovic; Maria De Santis; Lidia P Ananieva; Simone Negrini; David Launay; Valeria Riccieri; Andra Balanescu; Ana Maria Gheorghiu; Christina Bergmann; Luc Mouthon; Vanessa Smith; Addolorata Corrado; Mette Mogensen; Martin Aringer; Branimir Anić; Sule Yavuz; Julia Spierings

doi:10.1016/j.jaut.2026.103555

Beyond screening for pulmonary arterial hypertension: The DETECT score is a potential promising prediction tool for all-cause mortality in systemic sclerosis: Analysis from the EUSTAR database

Florian Käs
, Muriel Elhai
, Mike O Becker
, Rucsandra Dobrota
, Carina Mihai
, Gesa Sauer
, Lorenzo Tofani
, Radim Bečvář
, Simona Rednic
, Patricia E Carreira
, Gábor Kumánovics
, Paolo Airò
, Ulf Mueller-Ladner
, Francesco Del Galdo
, Ana-Maria Ramazan
, Mickaël Martin
, Carmen-Pilar Simeón-Aznar
, Magda Parvu
, Nicoletta Del Papa
, Anna-Maria Hoffmann-Vold

Oliver Distler, Cosimo Bruni^*, Silvia Bellando-Randone, Ulrich Andreas Walker, Florenzo Iannone, Yannick Allanore, Michele Iudici, Elisabetta Zanatta, Gianluca Moroncini, Mislav Radic, Nico Hunzelmann, Luca Idolazzi, Joerg Henes, Bojana Stamenkovic, Maria De Santis, Lidia P Ananieva, Simone Negrini, David Launay, Valeria Riccieri, Andra Balanescu, Ana Maria Gheorghiu, Christina Bergmann, Luc Mouthon, Vanessa Smith, Addolorata Corrado, Mette Mogensen, Martin Aringer, Branimir Anić, Sule Yavuz, Julia Spierings,

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

BACKGROUND: Systemic sclerosis (SSc) is characterized by an increased mortality. Various mortality risk factors are included in the DETECT algorithm, a screening tool for SSc-associated pulmonary arterial hypertension. We tested the DETECT score as a predictor of all-cause mortality in SSc.

METHODS: SSc patients from the European Scleroderma Trial And Research (EUSTAR) cohort, with available data for calculating the DETECT and the SCOpE (currently proposed risk algorithm) scores and follow-up were included. Patients from the University Hospital Zurich served as derivation cohort, the remaining EUSTAR patients formed the validation cohort. Uni- and multivariable Cox regression tested the DETECT score as a predictor of mortality. A time-dependent ROC curve analysis was used to assess predictive accuracy (at 1, 3, and 5 years), and to derive and validate optimal cutoffs.

RESULTS: The derivation cohort (n = 605) showed less cardio-pulmonary and diffuse cutaneous involvements, but longer follow-up and higher mortality than the validation cohort (n = 1017). The DETECT score independently predicted mortality in both cohorts, even after excluding pulmonary hypertension patients. Time-dependent ROC analysis showed excellent predictive accuracy for mortality (AUC>0.85) in the derivation cohort, non-inferior to the SCOpE score. In the validation cohort, a moderate-to-good performance for 1-year mortality was retained. A DETECT score>40 demonstrated strong performance (sensitivity≥0.68; specificity≥0.83) in the derivation, and performed moderately in the validation cohort (sensitivity = 0.54; specificity = 0.71).

CONCLUSION: The DETECT score robustly predicts all-cause mortality in SSc across phenotypically different cohorts. A DETECT score>40 may refine risk stratification, guiding tighter monitoring and management. Further validation over 1-year outcomes is warranted.

Original language	English
Article number	103555
Number of pages	10
Journal	Journal of Autoimmunity
Volume	160
Early online date	20 Apr 2026
DOIs	https://doi.org/10.1016/j.jaut.2026.103555
Publication status	Published - May 2026

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1-s2.0-S0896841126000338-mainFinal published version, 1.51 MBLicence: CC BY

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Käs, F., Elhai, M., Becker, M. O., Dobrota, R., Mihai, C., Sauer, G., Tofani, L., Bečvář, R., Rednic, S., Carreira, P. E., Kumánovics, G., Airò, P., Mueller-Ladner, U., Del Galdo, F., Ramazan, A.-M., Martin, M., Simeón-Aznar, C.-P., Parvu, M., Del Papa, N. (2026). Beyond screening for pulmonary arterial hypertension: The DETECT score is a potential promising prediction tool for all-cause mortality in systemic sclerosis: Analysis from the EUSTAR database. Journal of Autoimmunity, 160, Article 103555. https://doi.org/10.1016/j.jaut.2026.103555

@article{5a120f14502a40e1b175b6892f8544da,

title = "Beyond screening for pulmonary arterial hypertension: The DETECT score is a potential promising prediction tool for all-cause mortality in systemic sclerosis: Analysis from the EUSTAR database",

abstract = "BACKGROUND: Systemic sclerosis (SSc) is characterized by an increased mortality. Various mortality risk factors are included in the DETECT algorithm, a screening tool for SSc-associated pulmonary arterial hypertension. We tested the DETECT score as a predictor of all-cause mortality in SSc.METHODS: SSc patients from the European Scleroderma Trial And Research (EUSTAR) cohort, with available data for calculating the DETECT and the SCOpE (currently proposed risk algorithm) scores and follow-up were included. Patients from the University Hospital Zurich served as derivation cohort, the remaining EUSTAR patients formed the validation cohort. Uni- and multivariable Cox regression tested the DETECT score as a predictor of mortality. A time-dependent ROC curve analysis was used to assess predictive accuracy (at 1, 3, and 5 years), and to derive and validate optimal cutoffs.RESULTS: The derivation cohort (n = 605) showed less cardio-pulmonary and diffuse cutaneous involvements, but longer follow-up and higher mortality than the validation cohort (n = 1017). The DETECT score independently predicted mortality in both cohorts, even after excluding pulmonary hypertension patients. Time-dependent ROC analysis showed excellent predictive accuracy for mortality (AUC>0.85) in the derivation cohort, non-inferior to the SCOpE score. In the validation cohort, a moderate-to-good performance for 1-year mortality was retained. A DETECT score>40 demonstrated strong performance (sensitivity≥0.68; specificity≥0.83) in the derivation, and performed moderately in the validation cohort (sensitivity = 0.54; specificity = 0.71).CONCLUSION: The DETECT score robustly predicts all-cause mortality in SSc across phenotypically different cohorts. A DETECT score>40 may refine risk stratification, guiding tighter monitoring and management. Further validation over 1-year outcomes is warranted.",

author = "Florian K{\"a}s and Muriel Elhai and Becker, \{Mike O\} and Rucsandra Dobrota and Carina Mihai and Gesa Sauer and Lorenzo Tofani and Radim Be{\v c}v{\'a}{\v r} and Simona Rednic and Carreira, \{Patricia E\} and G{\'a}bor Kum{\'a}novics and Paolo Air{\`o} and Ulf Mueller-Ladner and \{Del Galdo\}, Francesco and Ana-Maria Ramazan and Micka{\"e}l Martin and Carmen-Pilar Sime{\'o}n-Aznar and Magda Parvu and \{Del Papa\}, Nicoletta and Anna-Maria Hoffmann-Vold and Oliver Distler and Cosimo Bruni and Silvia Bellando-Randone and Walker, \{Ulrich Andreas\} and Florenzo Iannone and Yannick Allanore and Michele Iudici and Elisabetta Zanatta and Gianluca Moroncini and Mislav Radic and Nico Hunzelmann and Luca Idolazzi and Joerg Henes and Bojana Stamenkovic and \{De Santis\}, Maria and Ananieva, \{Lidia P\} and Simone Negrini and David Launay and Valeria Riccieri and Andra Balanescu and Gheorghiu, \{Ana Maria\} and Christina Bergmann and Luc Mouthon and Vanessa Smith and Addolorata Corrado and Mette Mogensen and Martin Aringer and Branimir Ani{\'c} and Sule Yavuz and Julia Spierings",

note = "Publisher Copyright: {\textcopyright} 2026 The Authors",

year = "2026",

month = may,

doi = "10.1016/j.jaut.2026.103555",

language = "English",

volume = "160",

journal = "Journal of Autoimmunity",

issn = "0896-8411",

publisher = "Academic Press",

}

Käs, F, Elhai, M, Becker, MO, Dobrota, R, Mihai, C, Sauer, G, Tofani, L, Bečvář, R, Rednic, S, Carreira, PE, Kumánovics, G, Airò, P, Mueller-Ladner, U, Del Galdo, F, Ramazan, A-M, Martin, M, Simeón-Aznar, C-P, Parvu, M, Del Papa, N, Hoffmann-Vold, A-M, Distler, O, Bruni, C, Bellando-Randone, S, Walker, UA, Iannone, F, Allanore, Y, Iudici, M, Zanatta, E, Moroncini, G, Radic, M, Hunzelmann, N, Idolazzi, L, Henes, J, Stamenkovic, B, De Santis, M, Ananieva, LP, Negrini, S, Launay, D, Riccieri, V, Balanescu, A, Gheorghiu, AM, Bergmann, C, Mouthon, L, Smith, V, Corrado, A, Mogensen, M, Aringer, M, Anić, B, Yavuz, S, Spierings, J 2026, 'Beyond screening for pulmonary arterial hypertension: The DETECT score is a potential promising prediction tool for all-cause mortality in systemic sclerosis: Analysis from the EUSTAR database', Journal of Autoimmunity, vol. 160, 103555. https://doi.org/10.1016/j.jaut.2026.103555

Beyond screening for pulmonary arterial hypertension: The DETECT score is a potential promising prediction tool for all-cause mortality in systemic sclerosis: Analysis from the EUSTAR database. / Käs, Florian; Elhai, Muriel; Becker, Mike O et al.
In: Journal of Autoimmunity, Vol. 160, 103555, 05.2026.

Research output: Contribution to journal › Article › Academic › peer-review

TY - JOUR

T1 - Beyond screening for pulmonary arterial hypertension

T2 - The DETECT score is a potential promising prediction tool for all-cause mortality in systemic sclerosis: Analysis from the EUSTAR database

AU - Käs, Florian

AU - Elhai, Muriel

AU - Becker, Mike O

AU - Dobrota, Rucsandra

AU - Mihai, Carina

AU - Sauer, Gesa

AU - Tofani, Lorenzo

AU - Bečvář, Radim

AU - Rednic, Simona

AU - Carreira, Patricia E

AU - Kumánovics, Gábor

AU - Airò, Paolo

AU - Mueller-Ladner, Ulf

AU - Del Galdo, Francesco

AU - Ramazan, Ana-Maria

AU - Martin, Mickaël

AU - Simeón-Aznar, Carmen-Pilar

AU - Parvu, Magda

AU - Del Papa, Nicoletta

AU - Hoffmann-Vold, Anna-Maria

AU - Distler, Oliver

AU - Bruni, Cosimo

AU - Bellando-Randone, Silvia

AU - Walker, Ulrich Andreas

AU - Iannone, Florenzo

AU - Allanore, Yannick

AU - Iudici, Michele

AU - Zanatta, Elisabetta

AU - Moroncini, Gianluca

AU - Radic, Mislav

AU - Hunzelmann, Nico

AU - Idolazzi, Luca

AU - Henes, Joerg

AU - Stamenkovic, Bojana

AU - De Santis, Maria

AU - Ananieva, Lidia P

AU - Negrini, Simone

AU - Launay, David

AU - Riccieri, Valeria

AU - Balanescu, Andra

AU - Gheorghiu, Ana Maria

AU - Bergmann, Christina

AU - Mouthon, Luc

AU - Smith, Vanessa

AU - Corrado, Addolorata

AU - Mogensen, Mette

AU - Aringer, Martin

AU - Anić, Branimir

AU - Yavuz, Sule

AU - Spierings, Julia

PY - 2026/5

Y1 - 2026/5

N2 - BACKGROUND: Systemic sclerosis (SSc) is characterized by an increased mortality. Various mortality risk factors are included in the DETECT algorithm, a screening tool for SSc-associated pulmonary arterial hypertension. We tested the DETECT score as a predictor of all-cause mortality in SSc.METHODS: SSc patients from the European Scleroderma Trial And Research (EUSTAR) cohort, with available data for calculating the DETECT and the SCOpE (currently proposed risk algorithm) scores and follow-up were included. Patients from the University Hospital Zurich served as derivation cohort, the remaining EUSTAR patients formed the validation cohort. Uni- and multivariable Cox regression tested the DETECT score as a predictor of mortality. A time-dependent ROC curve analysis was used to assess predictive accuracy (at 1, 3, and 5 years), and to derive and validate optimal cutoffs.RESULTS: The derivation cohort (n = 605) showed less cardio-pulmonary and diffuse cutaneous involvements, but longer follow-up and higher mortality than the validation cohort (n = 1017). The DETECT score independently predicted mortality in both cohorts, even after excluding pulmonary hypertension patients. Time-dependent ROC analysis showed excellent predictive accuracy for mortality (AUC>0.85) in the derivation cohort, non-inferior to the SCOpE score. In the validation cohort, a moderate-to-good performance for 1-year mortality was retained. A DETECT score>40 demonstrated strong performance (sensitivity≥0.68; specificity≥0.83) in the derivation, and performed moderately in the validation cohort (sensitivity = 0.54; specificity = 0.71).CONCLUSION: The DETECT score robustly predicts all-cause mortality in SSc across phenotypically different cohorts. A DETECT score>40 may refine risk stratification, guiding tighter monitoring and management. Further validation over 1-year outcomes is warranted.

AB - BACKGROUND: Systemic sclerosis (SSc) is characterized by an increased mortality. Various mortality risk factors are included in the DETECT algorithm, a screening tool for SSc-associated pulmonary arterial hypertension. We tested the DETECT score as a predictor of all-cause mortality in SSc.METHODS: SSc patients from the European Scleroderma Trial And Research (EUSTAR) cohort, with available data for calculating the DETECT and the SCOpE (currently proposed risk algorithm) scores and follow-up were included. Patients from the University Hospital Zurich served as derivation cohort, the remaining EUSTAR patients formed the validation cohort. Uni- and multivariable Cox regression tested the DETECT score as a predictor of mortality. A time-dependent ROC curve analysis was used to assess predictive accuracy (at 1, 3, and 5 years), and to derive and validate optimal cutoffs.RESULTS: The derivation cohort (n = 605) showed less cardio-pulmonary and diffuse cutaneous involvements, but longer follow-up and higher mortality than the validation cohort (n = 1017). The DETECT score independently predicted mortality in both cohorts, even after excluding pulmonary hypertension patients. Time-dependent ROC analysis showed excellent predictive accuracy for mortality (AUC>0.85) in the derivation cohort, non-inferior to the SCOpE score. In the validation cohort, a moderate-to-good performance for 1-year mortality was retained. A DETECT score>40 demonstrated strong performance (sensitivity≥0.68; specificity≥0.83) in the derivation, and performed moderately in the validation cohort (sensitivity = 0.54; specificity = 0.71).CONCLUSION: The DETECT score robustly predicts all-cause mortality in SSc across phenotypically different cohorts. A DETECT score>40 may refine risk stratification, guiding tighter monitoring and management. Further validation over 1-year outcomes is warranted.

U2 - 10.1016/j.jaut.2026.103555

DO - 10.1016/j.jaut.2026.103555

M3 - Article

C2 - 42013635

SN - 0896-8411

VL - 160

JO - Journal of Autoimmunity

JF - Journal of Autoimmunity

M1 - 103555

ER -

Beyond screening for pulmonary arterial hypertension: The DETECT score is a potential promising prediction tool for all-cause mortality in systemic sclerosis: Analysis from the EUSTAR database

Abstract

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