Predictors of disease worsening defined by progression of organ damage in diffuse systemic sclerosis: a European Scleroderma Trials and Research (EUSTAR) analysis


Por: Becker, M, Graf, N, Sauter, R, Curram, J, Denton, C, Khanna, D, Pena, J, Pope, J, Distler, O, Matucci-Cerinic, M, Guiducci, S, Walker, U, Jaeger, V, Bannert, B, Lapadula, G, Becvarare, R, Cutolo, M, Valentini, G, Siegert, E, Rednic, S, Allanore, Y, Montecucco, C, Carreira, P, Novak, S, Czirjak, L, Varju, C, Chizzolini, C, Allai, D, Kucharz, E, Cozzi, F, Rozman, B, Mallia, C, Gabrielli, A, Bancel, D, Airo, P, Hesselstrand, R, Martinovic, D, Balbir-Gurman, A, Braun-Moscovici, Y, Hunzelmann, N, Pellerito, R, Caramaschi, P, Black, C, Damjanov, N, Henes, J, Santamaria, V, Heitmann, S, Seidel, M, Da Silva, J, Stamenkovic, B, Selmi, C, Tikly, M, Denisov, L, Muller-Ladner, U, Engelhart, M, Hachulla, E, Riccieri, V, Ionescu, R, Mihai, C, Sunderkotter, C, Kuhn, A, Schett, G, Distler, J, Meroni, P, Ingegnoli, F, Mouthon, L, De Keyser, F, Smith, V, Cantatore, F, Corrado, A, Ullman, S, Iversen, L, Pozzi, M, Eyerich, K, Hein, R, Knott, E, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Alegre-Sancho, J, Krummel-Lorenz, B, Saar, P, Aringer, M, Gunther, C, Anne, E, Westhovens, R, De Langhe, E, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Uprus, M, Otsa, K, Yavuz, S, Radominski, S, Muller, C, Azevedo, V, Popa, S, Zenone, T, Stebbings, S, Highton, J, Mathieu, A, Vacca, A, Stamp, L, Chapman, P, O'Donnell, J, Solanki, K, Doube, A, Veale, D, O'Rourke, M, Loyo, E, Li, M, Rosato, E, Amoroso, A, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, C, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Visalli, E, Benenati, A, Amato, G, Ancuta, C, Chirieac, R, Villiger, P, Adler, S, Dan, D, Lefebvre, P, Rubio, S, Exposito, M, Sibilia, J, Chatelus, E, Gottenberg, J, Chifflot, H, Litinsky, I, Del Galdo, F, Venalis, A, Saketkoo, L, Lasky, J, Kerzberg, E, Montoya, F, Cosentino, V, Limonta, M, Brucato, A, Lupi, E, Spertini, F, Ribi, C, Buss, G, Martin, T, Guffroy, A, Poindron, V, Chung, L, Schmeiser, T, Zebryk, P, Riso, N, Riemekasten, G, Rezus, E, Puttini, P and EUSTAR Collaborators

Publicada: 1 sep 2019
Resumen:
Objectives Mortality and worsening of organ function are desirable endpoints for clinical trials in systemic sclerosis (SSc). The aim of this study was to identify factors that allow enrichment of patients with these endpoints, in a population of patients from the European Scleroderma Trials and Research group database. Methods Inclusion criteria were diagnosis of diffuse SSc and follow-up over 12 +/- 3 months. Disease worsening/organ progression was fulfilled if any of the following events occurred: new renal crisis; decrease of lung or heart function; new echocardiography-suspected pulmonary hypertension or death. In total, 42 clinical parameters were chosen as predictors for the analysis by using (1) imputation of missing data on the basis of multivariate imputation and (2) least absolute shrinkage and selection operator regression. Results Of 1451 patients meeting the inclusion criteria, 706 had complete data on outcome parameters and were included in the analysis. Of the 42 outcome predictors, eight remained in the final regression model. There was substantial evidence for a strong association between disease progression and age, active digital ulcer (DU), lung fibrosis, muscle weakness and elevated C-reactive protein (CRP) level. Active DU, CRP elevation, lung fibrosis and muscle weakness were also associated with a significantly shorter time to disease progression. A bootstrap validation step with 10 000 repetitions successfully validated the model. Conclusions The use of the predictive factors presented here could enable cohort enrichment with patients at risk for overall disease worsening in SSc clinical trials.

Filiaciones:
Becker, M:
 Univ Hosp Zurich, Dept Rheumatol, CH-8091 Zurich, Switzerland

 Univ Hosp Zurich, Ctr Expt Rheumatol, Zurich, Switzerland

Graf, N:
 Graf Biostat, Winterthur, Switzerland

Sauter, R:
 Univ Oxford, Nuffield Dept Med, Big Data Inst, Li Ka Shing Ctr Hlth Informat & Discovery, Oxford, England

Curram, J:
 Bayer PLC, Data Sci & Analyt, Reading, Berks, England

Denton, C:
 UCL Div Med, Royal Free Campus, London, England

Khanna, D:
 Univ Michigan, Dept Internal Med, Div Rheumatol, Scleroderma Program, Ann Arbor, MI 48109 USA

Pena, J:
 Bayer US LLC, Whippany, NJ USA

Pope, J:
 Univ Western Ontario, St Josephs Hlth Care, Dept Med, Div Rheumatol, London, ON, Canada

Distler, O:
 Univ Hosp Zurich, Dept Rheumatol, CH-8091 Zurich, Switzerland

 Univ Hosp Zurich, Ctr Expt Rheumatol, Zurich, Switzerland

Matucci-Cerinic, M:
 Univ Florence, Dept Expt & Clin Med, Florence, Italy

 Univ Florence, Florence, Italy

Guiducci, S:
 Univ Florence, Florence, Italy

Walker, U:
 Univ Hosp Basel, Basel, Switzerland

Jaeger, V:
 Univ Hosp Basel, Basel, Switzerland

Bannert, B:
 Univ Hosp Basel, Basel, Switzerland

Lapadula, G:
 Univ Bari, Sch Med, Bari, Italy

Becvarare, R:
 Charles Univ Prague, Med Sch 1, Prague, Czech Republic

Cutolo, M:
 Univ Genoa, Genoa, Italy

Valentini, G:
 Policlin UO Reumatol, Naples, Italy

Siegert, E:
 Charite, Berlin, Germany

Rednic, S:
 Univ Med & Pharm Iuliu Hatieganu Cluj, Cluj Napoca, Romania

Allanore, Y:
 Paris Descartes Univ, Cochin Hosp, Sorbonne Paris Cite, Rheumatol Dept A, Paris, France

 Univ Cochin Hosp, Paris, France

Montecucco, C:
 IRCCS Policlin S Matteo, Pavia, Italy

Carreira, P:
 Hosp 12 Octubre, Madrid, Spain

Novak, S:
 KBC Rijeka, Rijeka, Croatia

Czirjak, L:
 Univ Pecs, Pecs, Hungary

Varju, C:
 Univ Pecs, Pecs, Hungary

Chizzolini, C:
 Univ Hosp Geneva, Geneva, Switzerland

Allai, D:
 Univ Hosp Geneva, Geneva, Switzerland

Kucharz, E:
 Med Univ Silesia, Katowice, Poland

Cozzi, F:
 Univ Padua, Padua, Italy

Rozman, B:
 Univ Med Ctr Ljubljana, Ljubljana, Slovenia

Mallia, C:
 Stella Maris, Balzan, Malta

Gabrielli, A:
 Univ Ancona, Univ Politecn Marche Polo Didatt, Ist Clin Med Gen Ematol & Immunol Clin, Ancona, Italy

Bancel, D:
 Hop St Louis, Paris, France

Airo, P:
 Spedali Civili Brescia, Serv Reumatol Allergol & Immunol Clin, Brescia, Italy

Hesselstrand, R:
 Lund Univ Hosp, Lund, Sweden

Martinovic, D:
 Clin Hosp Split, Split, Croatia

Balbir-Gurman, A:
 Rambam Hlth Care Campus, B Shine Rheumatol Inst, Haifa, Israel

Braun-Moscovici, Y:
 Rambam Hlth Care Campus, B Shine Rheumatol Inst, Haifa, Israel

Hunzelmann, N:
 Univ Haut Klin Koln, Cologne, Germany

Pellerito, R:
 Osped Mauriziano Umberto 1, Turin, Italy

Caramaschi, P:
 Univ Verona, Verona, Italy

Black, C:
 Royal Free & Univ Coll London Med Sch, London, England

Damjanov, N:
 Inst Rheumatol, Belgrade, Serbia

 Inst Rheumatol, Podgorica, Montenegro

Henes, J:
 Med Univ Klin Abt II, Tubingen, Germany

Santamaria, V:
 Rheumatol Granollers Gen Hosp, Barcelona, Spain

Heitmann, S:
 Marienhosp Stuttgart, Stuttgart, Germany

Seidel, M:
 Med Univ Poliklin, Bonn, Germany

Da Silva, J:
 Univ Coimbra, Coimbra, Portugal

Stamenkovic, B:
 Inst Prevent Treatment & Rehabil Rheumat & Cardio, Nish, Serbia

 Inst Prevent Treatment & Rehabil Rheumat & Cardio, Podgorica, Montenegro

Selmi, C:
 Univ Milan, Milan, Italy

Tikly, M:
 Chris Hani Baragwanath Hosp, Johannesburg, South Africa

 Univ Witwatersrand, Johannesburg, South Africa

Denisov, L:
 VA Nasonova Inst Rheumatol, Moscow, Russia

Muller-Ladner, U:
 Kerckhoff Clin Bad Nauheim, Bad Nauheim, Germany

Engelhart, M:
 Univ Hosp Gentofte, Hellerup, Denmark

Hachulla, E:
 Hop Claude Huriez, Lille, France

Riccieri, V:
 Sapienza Univ Roma, Rome, Italy

Ionescu, R:
 Carol Davila Univ Med & Pharm, St Maria Hosp, Bucharest, Romania

Mihai, C:
 Ion Cantacuzino Clin Hosp, Bucharest, Romania

Sunderkotter, C:
 Univ Munster, Munster, Germany

Kuhn, A:
 Univ Munster, Munster, Germany

Schett, G:
 Univ Klinikum Erlangen, Erlangen, Germany

Distler, J:
 Univ Klinikum Erlangen, Erlangen, Germany

Meroni, P:
 Univ Milan, Ist Gaetano Pini, Milan, Italy

Ingegnoli, F:
 Univ Milan, Ist Gaetano Pini, Milan, Italy

Mouthon, L:
 Hop Cochin, Paris, France

De Keyser, F:
 Univ Ghent, Ghent, Belgium

Smith, V:
 Univ Ghent, Ghent, Belgium

Cantatore, F:
 Univ Foggia, UO Reumatol, Osped Col DAvanzo, Foggia, Italy

Corrado, A:
 Univ Foggia, UO Reumatol, Osped Col DAvanzo, Foggia, Italy

Ullman, S:
 Univ Hosp Copenhagen, Copenhagen, Denmark

Iversen, L:
 Univ Hosp Copenhagen, Copenhagen, Denmark

Pozzi, M:
 Osped San Gerardo, Monza, Italy

Eyerich, K:
 Tech Univ Munich, Munich, Germany

Hein, R:
 Tech Univ Munich, Munich, Germany

Knott, E:
 Tech Univ Munich, Munich, Germany

Wiland, P:
 Wroclaw Univ Med, Wroclaw, Poland

Szmyrka-Kaczmarek, M:
 Wroclaw Univ Med, Wroclaw, Poland

Sokolik, R:
 Wroclaw Univ Med, Wroclaw, Poland

Morgiel, E:
 Wroclaw Univ Med, Wroclaw, Poland

Madej, M:
 Wroclaw Univ Med, Wroclaw, Poland

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 Hosp Univ Dr Peset, Valencia, Spain

Krummel-Lorenz, B:
 Endokrinol Frankfurt, Frankfurt, Germany

Saar, P:
 Endokrinol Frankfurt, Frankfurt, Germany

Aringer, M:
 Carl Gustav Carus Tech Univ Dresden, Univ Med Ctr, Dresden, Germany

Gunther, C:
 Carl Gustav Carus Tech Univ Dresden, Univ Med Ctr, Dresden, Germany

Anne, E:
 Carl Gustav Carus Tech Univ Dresden, Univ Med Ctr, Dresden, Germany

Westhovens, R:
 Univ Hosp Leuven, Skeletal Biol & Engn Res Ctr, Leuven, Belgium

De Langhe, E:
 Univ Hosp Leuven, Skeletal Biol & Engn Res Ctr, Leuven, Belgium

Lenaerts, J:
 Univ Hosp Leuven, Skeletal Biol & Engn Res Ctr, Leuven, Belgium

Anic, B:
 Univ Zagreb, Sch Med, Univ Hosp Ctr Zagreb, Zagreb, Croatia

Baresic, M:
 Univ Zagreb, Sch Med, Univ Hosp Ctr Zagreb, Zagreb, Croatia

Mayer, M:
 Univ Zagreb, Sch Med, Univ Hosp Ctr Zagreb, Zagreb, Croatia

Uprus, M:
 East Tallin Cent Hosp, Tallinn, Estonia

Otsa, K:
 East Tallin Cent Hosp, Tallinn, Estonia

Yavuz, S:
 Univ Marmara, Istanbul, Turkey

Radominski, S:
 Univ Fed Parana, Hosp Clin, Curitiba, Parana, Brazil

Muller, C:
 Univ Fed Parana, Hosp Clin, Curitiba, Parana, Brazil

Azevedo, V:
 Univ Fed Parana, Hosp Clin, Curitiba, Parana, Brazil

Popa, S:
 Republican Clin Hosp, Kishinev, Moldova

Zenone, T:
 Unit Internal Med, Valence, France

Stebbings, S:
 Dunedin Sch Med, Dunedin, New Zealand

Highton, J:
 Dunedin Sch Med, Dunedin, New Zealand

Mathieu, A:
 Univ Cagliari, Policlin Univ, Chair Rheumatol 2, Cagliari, Italy

Vacca, A:
 Univ Cagliari, Policlin Univ, Chair Rheumatol 2, Cagliari, Italy

Stamp, L:
 Univ Otago, Christchurch, New Zealand

Chapman, P:
 Univ Otago, Christchurch, New Zealand

O'Donnell, J:
 Univ Otago, Christchurch, New Zealand

Solanki, K:
 Waikato Univ Hosp, Hamilton, New Zealand

Doube, A:
 Waikato Univ Hosp, Hamilton, New Zealand

Veale, D:
 St Vincents Univ Hosp, Dublin, Ireland

O'Rourke, M:
 St Vincents Univ Hosp, Dublin, Ireland

Loyo, E:
 Hosp Reg Univ Jose Ma Cabral & Baez, Clin Corominas, Santiago, Dominican Rep

Li, M:
 Peking Union Med Coll Hosp, West Campus, Beijing, Peoples R China

Rosato, E:
 Sapienza Univ Roma, Univ La Sapienza, Policlin Umberto 1, Rome, Italy

Amoroso, A:
 Sapienza Univ Roma, Univ La Sapienza, Policlin Umberto 1, Rome, Italy

Gigante, A:
 Sapienza Univ Roma, Univ La Sapienza, Policlin Umberto 1, Rome, Italy

Oksel, F:
 Ege Univ, Izmir, Turkey

Yargucu, F:
 Ege Univ, Izmir, Turkey

Tanaseanu, C:
 Clin Emergency Hosp St Pantelimon, Bucharest, Romania

Popescu, M:
 Clin Emergency Hosp St Pantelimon, Bucharest, Romania

Dumitrascu, A:
 Clin Emergency Hosp St Pantelimon, Bucharest, Romania

Tiglea, I:
 Clin Emergency Hosp St Pantelimon, Bucharest, Romania

Foti, R:
 AOU Policlin VE Catania Ctr Riferimento Reg Malat, AOU Policlin Vittorio Emanuele UO Reumatol, Catania, Italy

Visalli, E:
 AOU Policlin VE Catania Ctr Riferimento Reg Malat, AOU Policlin Vittorio Emanuele UO Reumatol, Catania, Italy

Benenati, A:
 AOU Policlin VE Catania Ctr Riferimento Reg Malat, AOU Policlin Vittorio Emanuele UO Reumatol, Catania, Italy

Amato, G:
 AOU Policlin VE Catania Ctr Riferimento Reg Malat, AOU Policlin Vittorio Emanuele UO Reumatol, Catania, Italy

Ancuta, C:
 Univ Med & Pharm, Rehabil Hosp, European Ctr Translat Res, GR T Popa Ctr Biomed Res, Iasi, Romania

Chirieac, R:
 Univ Med & Pharm, Rehabil Hosp, European Ctr Translat Res, GR T Popa Ctr Biomed Res, Iasi, Romania

Villiger, P:
 Univ Bern, Bern, Switzerland

Adler, S:
 Univ Bern, Bern, Switzerland

Dan, D:
 Univ Bern, Bern, Switzerland

Lefebvre, P:
 Hosp Univ Sanchinarro, Madrid, Spain

Rubio, S:
 Hosp Univ Sanchinarro, Madrid, Spain

Exposito, M:
 Hosp Univ Sanchinarro, Madrid, Spain

Sibilia, J:
 Univ Hosp Strasbourg, Hop Hautepierre, Strasbourg, France

Chatelus, E:
 Univ Hosp Strasbourg, Hop Hautepierre, Strasbourg, France

Gottenberg, J:
 Univ Hosp Strasbourg, Hop Hautepierre, Strasbourg, France

Chifflot, H:
 Univ Hosp Strasbourg, Hop Hautepierre, Strasbourg, France

Litinsky, I:
 Tel Aviv Sourasky Med Ctr, Tel Aviv, Israel

Del Galdo, F:
 Univ Leeds, Chapel Allerton Hosp, Leeds, W Yorkshire, England

Venalis, A:
 Vilnius Univ, State Res Inst Innovat Med, Vilnius, Lithuania

Saketkoo, L:
 Tulane Univ, Med Ctr, Scleroderma & Sarcoidosis Patient Care & Res Ctr, New Orleans, LA 70118 USA

Lasky, J:
 Tulane Univ, Med Ctr, Scleroderma & Sarcoidosis Patient Care & Res Ctr, New Orleans, LA 70118 USA

Kerzberg, E:
 Univ Buenos Aires, Ramos Mejia Hosp, Sch Med, Osteoarticular Dis & Osteoporosis Ctr,Pharmacol &, Buenos Aires, DF, Argentina

Montoya, F:
 Univ Buenos Aires, Ramos Mejia Hosp, Sch Med, Osteoarticular Dis & Osteoporosis Ctr,Pharmacol &, Buenos Aires, DF, Argentina

Cosentino, V:
 Univ Buenos Aires, Ramos Mejia Hosp, Sch Med, Osteoarticular Dis & Osteoporosis Ctr,Pharmacol &, Buenos Aires, DF, Argentina

Limonta, M:
 Osped Riuniti Bergamo, USSD Reumatol, Bergamo, Italy

Brucato, A:
 Osped Riuniti Bergamo, USSD Reumatol, Bergamo, Italy

Lupi, E:
 Osped Riuniti Bergamo, USSD Reumatol, Bergamo, Italy

Spertini, F:
 Dept Rheumatol Clin Immunol & Allergy, Lausanne, Switzerland

Ribi, C:
 Dept Rheumatol Clin Immunol & Allergy, Lausanne, Switzerland

Buss, G:
 Dept Rheumatol Clin Immunol & Allergy, Lausanne, Switzerland

Martin, T:
 Nouvel Hop Civil, Strasbourg, France

Guffroy, A:
 Nouvel Hop Civil, Strasbourg, France

Poindron, V:
 Nouvel Hop Civil, Strasbourg, France

Chung, L:
 Stanford Univ, Sch Med, Stanford, CA 94305 USA

Schmeiser, T:
 Krankenhaus St Josef, Wuppertal, Germany

Zebryk, P:
 Poznan Univ Med Sci, Poznan, Poland

Riso, N:
 Ctr Hosp Lisboa Cent, Hosp Curry Cabral, Unidade Doencas Autoimunes, Lisbon, Portugal

Riemekasten, G:
 Univ Klin Lubeck, Lubeck, Germany

Rezus, E:
 Univ Med & Pharm GR T Popa Iasi, Rehabil Hosp, Iasi, Romania

Puttini, P:
 Univ Hosp Luigi Sacco, Milan, Italy
ISSN: 00034967





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BMJ PUBLISHING GROUP, BRITISH MED ASSOC HOUSE, TAVISTOCK SQUARE, LONDON WC1H 9JR, ENGLAND, Reino Unido
Tipo de documento: Article
Volumen: 78 Número: 9
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