Stratification in systemic sclerosis ...
Document type :
Article dans une revue scientifique: Article original
PMID :
Permalink :
Title :
Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort.
Author(s) :
Elhai, Muriel [Auteur]
University hospital of Zurich [Zurich]
Hôpital Cochin [AP-HP]
Sritharan, Nanthara [Auteur]
Centre Hospitalier de Saint-Denis [Ile-de-France]
Boubaya, Marouane [Auteur]
Centre Hospitalier de Saint-Denis [Ile-de-France]
Balbir-Gurman, Alexandra [Auteur]
Rappaport faculty of Medicine
Siegert, Elise [Auteur]
Charité - UniversitätsMedizin = Berlin University Medicine
Hachulla, Eric [Auteur]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
De Vries-Bouwstra, Jeska [Auteur]
Leiden University Medical Center [LUMC]
Riemekasten, Gabriela [Auteur]
Universität zu Lübeck = University of Lübeck [Lübeck]
Distler, Jörg H. W. [Auteur]
University Hospital Düsseldorf
Rosato, Edoardo [Auteur]
Università degli Studi di Roma "La Sapienza" = Sapienza University [Rome] [UNIROMA]
Del Galdo, Francesco [Auteur]
University of Leeds
Mendoza, Fabian A. [Auteur]
Jefferson (Philadelphia University + Thomas Jefferson University)
Furst, Daniel E. [Auteur]
De La Puente, Carlos [Auteur]
Hospital Universitario Ramón y Cajal [Madrid]
Hoffmann-Vold, Anna-Maria [Auteur]
Gabrielli, Armando [Auteur]
Polytechnic University of Marche / Università Politecnica delle Marche [UNIVPM]
Distler, Oliver [Auteur]
University hospital of Zurich [Zurich]
Bloch-Queyrat, Coralie [Auteur]
Centre Hospitalier de Saint-Denis [Ile-de-France]
Allanore, Yannick [Auteur]
Hôpital Cochin [AP-HP]
University hospital of Zurich [Zurich]
Hôpital Cochin [AP-HP]
Sritharan, Nanthara [Auteur]
Centre Hospitalier de Saint-Denis [Ile-de-France]
Boubaya, Marouane [Auteur]
Centre Hospitalier de Saint-Denis [Ile-de-France]
Balbir-Gurman, Alexandra [Auteur]
Rappaport faculty of Medicine
Siegert, Elise [Auteur]
Charité - UniversitätsMedizin = Berlin University Medicine
Hachulla, Eric [Auteur]

Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
De Vries-Bouwstra, Jeska [Auteur]
Leiden University Medical Center [LUMC]
Riemekasten, Gabriela [Auteur]
Universität zu Lübeck = University of Lübeck [Lübeck]
Distler, Jörg H. W. [Auteur]
University Hospital Düsseldorf
Rosato, Edoardo [Auteur]
Università degli Studi di Roma "La Sapienza" = Sapienza University [Rome] [UNIROMA]
Del Galdo, Francesco [Auteur]
University of Leeds
Mendoza, Fabian A. [Auteur]
Jefferson (Philadelphia University + Thomas Jefferson University)
Furst, Daniel E. [Auteur]
De La Puente, Carlos [Auteur]
Hospital Universitario Ramón y Cajal [Madrid]
Hoffmann-Vold, Anna-Maria [Auteur]
Gabrielli, Armando [Auteur]
Polytechnic University of Marche / Università Politecnica delle Marche [UNIVPM]
Distler, Oliver [Auteur]
University hospital of Zurich [Zurich]
Bloch-Queyrat, Coralie [Auteur]
Centre Hospitalier de Saint-Denis [Ile-de-France]
Allanore, Yannick [Auteur]
Hôpital Cochin [AP-HP]
Journal title :
The Lancet Rheumatology
Abbreviated title :
Lancet Rheumatol
Volume number :
4
Pages :
e785-e794
Publication date :
2024-01-25
ISSN :
2665-9913
English abstract : [en]
Background
The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous ...
Show more >Background The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15·4%) of 10 709 were male, 9062 (84·6%) were female, mean age was 54·4 (SD 13·8) years, and mean disease duration was 7·9 (SD 8·2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0·82, 95% CI 0·81–0·84 for cutaneous only vs 0·84, 0·82–0·85 for antibody only vs 0·84, 0·83–0·86 for combined) or for progression-free survival (0·70, 0·69–0·71 vs 0·71, 0·70–0·72 vs 0·71, 0·70–0·72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0·57, 0·46–0·71 for antibody only vs 0·29, 0·19–0·39 for cutaneous only) and disease progression (0·36, 0·29–0·46 vs 0·21, 0·14–0·28). The antibody-only model did better than the cutaneous-only model in predicting renal crisis (AUC 0·72, 0·70–0·74 for antibody only vs 0·66, 0·64–0·69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0·76, 0·75–0·77 vs 0·71, 0·70–0·72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Funding World Scleroderma Foundation.Show less >
Show more >Background The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15·4%) of 10 709 were male, 9062 (84·6%) were female, mean age was 54·4 (SD 13·8) years, and mean disease duration was 7·9 (SD 8·2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0·82, 95% CI 0·81–0·84 for cutaneous only vs 0·84, 0·82–0·85 for antibody only vs 0·84, 0·83–0·86 for combined) or for progression-free survival (0·70, 0·69–0·71 vs 0·71, 0·70–0·72 vs 0·71, 0·70–0·72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0·57, 0·46–0·71 for antibody only vs 0·29, 0·19–0·39 for cutaneous only) and disease progression (0·36, 0·29–0·46 vs 0·21, 0·14–0·28). The antibody-only model did better than the cutaneous-only model in predicting renal crisis (AUC 0·72, 0·70–0·74 for antibody only vs 0·66, 0·64–0·69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0·76, 0·75–0·77 vs 0·71, 0·70–0·72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Funding World Scleroderma Foundation.Show less >
Language :
Anglais
Audience :
Internationale
Popular science :
Non
Administrative institution(s) :
Université de Lille
Inserm
CHU Lille
Inserm
CHU Lille
Submission date :
2024-02-06T22:05:20Z
2024-03-04T13:06:57Z
2024-03-04T13:06:57Z