Abstract
Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.
Original language | English |
---|---|
Pages (from-to) | 754-763 |
Number of pages | 10 |
Journal | Annals of the Rheumatic Diseases |
Volume | 66 |
Issue number | 6 |
DOIs | |
State | Published - Jun 2007 |
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Clinical risk assessment of organ manifestations in systemic sclerosis : A report from the EULAR Scleroderma Trials and Research group database. / Walker, U. A.; Tyndall, A.; Czirják, L.; Denton, C.; Farge-Bancel, D.; Kowal-Bielecka, O.; Müller-Ladner, U.; Bocelli-Tyndall, C.; Matucci-Cerinic, M.; Riemekasten, Gabriela; Brückner, Claudia; Airó, Paolo; Scarsi, Mirko; Scorza, Raffaella; Beretta, Lorenzo; Cozzi, Franco; Tiso, Francesco; Vonk, M. C.; Van Den Hoogen, F. H.J.; Wigley, Fredrick M.; Hummers, Laura; Nevskaya, Tatjana; Ananieva, Lidia; Miniati, Irene; Tartaglia, Nicoletta; Lomater, Claudia; Balbir-Gurman, Alexandra; Braun-Moscovici, Yolanda; Bambara, Lisa Maria; Caramaschi, Paola; Valentini, Gabriele; Ruocco, Luigia; Krieg, Thomas; Hunzelmann, Nicolas; Varjú, Cecília; Carriera, Patricia E.; Joven, Beatriz; Iannone, Florenzo; Lapadula, Giovanni; Kahan, André; Allanore, Yannick; Gabrielli, Armando; Imperatore, Michele; Scheja, Agneta; Wollheim, Frank; Damjanov, Nemanja; Ostojic, Predrag; Saar, Petra; Tarner, Ingo H.; Kötter, Ina; Bombardieri, Stefano; Bazzichi, Laura; Del Papa, Nicoletta; Comina, Denise P.; Lo Monaco, Andrea; La Corte, Renato; Hachulla, Eric; Launay, David; Distler, Oliver; Ciurea, Adrian; Sierakowski, Stanislaw; Mitchell, Holly; Silver, Richard M.; Krasowska, Dorota; Michalska-Jakubus, Malgorzata; Tikly, Mohammed; Aboo, Nazrana; Worm, Margitta; Klaus, Pascal; Rovenský, Jozef; Lukáčová, Olga; Rozman, Blaz; Sipek, Alenka; Clemente-Coelho, Paulo; Shoenfeld, Yehuda; Langewitch, Pnina; Da Silva José, A. P.; Salvador, M. J.; Kuhn, Annegret; Erdmann, Gunilla; Bečvář, Radim; Friedl, Elke; Graninger, Winfried; Riccieri, Valeria; Caporali, Roberto; Montecucco, Carlomaurizio; Vlachoyiannopoulos, P.; Distler, Meike; Reich, Kristian; Majdan, Maria; Wielosz, Ewa; Rednic, Simona; Van Laar, Jacob M.; Heitmann, Stefan; Bruckner, Andreas; Himsel, Andrea; Riemann, Julia; Meyringer, Rotraud; Müller, Adelheid; Martinovic, Duska; Radic, Mislav; Sticherling, Michael; Szekanecz, Zoltan; Szücs, Gabriella; Giacomelli, Roberto; Marrelli, Alessandra; Stamenkovic, Bojana; Stankovic, Aleksandra; Aringer, Martin; Smolen, Josef S.; Kucharz, Eugene J.; Kotulska, Anna T.; Jablonska, Stefania; Blasczik, Maria; Jun, Jae Bum; Mallia, Carmel; Coleiro, Bernard; Santamaria, Vera Ortiz; Hinrichs, Ralf; Nielsen, Henrik; Cossutta, Roberta; Ionescu, Ruxandra; Opris, Daniela; Steinbrink, Kerstin; Grundt, Boris; Bajocchi, Gianluigi; Jiří, Štork; Lefebvre, Paloma García De La Peña; Zea Mendoza, Antonio C.; Ribi, Camillo; Chizzolini, Carlo; Wisłowska, Margaret; Novak, Srdan; Indiveri, Francesco; Jacobsen, Søren; Frandsen, Per Brown; Gorska, I. Zimmermann; Tore Gran, Jane; Midtvedt, Øyvind; Ramos, Filipa Oliveira; Rajcevska, Ljubinka Damjanovska; Bozinovski, Georgi; Schöffel, Dieter; Sunderkötter, Cord; Böhm, Markus; Morović-Vergles, Jadranka; Čulo, Melanie Ivana; Cutolo, Maurizio; Sulli, Alberto; Derk, Chris T.; Jimenez, Sergio A.; Siakka, Panagiota; Søndergaard, Klaus; Stengaard-Pedersen, Kristian; Cabane, Jean; Tiev, Kiet Phong; Mihai, Carina; Sfrent-Cornateanu, Roxana; Jendro, Michael; Tuvik, Piia; Antivalle, Marco; Randisi, Giovanna; Seidel, Matthias; Clarenbach, Ricarda; Simsek, Ismail; Dinc, Ayhan; Inanc, Murat; Capraru, Monica Sinziana; Capraru, Dorin; Bañegil, Inmaculada; Richter, Jutta; Alhasani, Saad; Földvari, Ivan; Pinto, Sandra; Brandão, Filipe; Mas, Antonio Juan.
In: Annals of the Rheumatic Diseases, Vol. 66, No. 6, 06.2007, p. 754-763.Research output: Contribution to journal › Article › peer-review
TY - JOUR
T1 - Clinical risk assessment of organ manifestations in systemic sclerosis
T2 - A report from the EULAR Scleroderma Trials and Research group database
AU - Walker, U. A.
AU - Tyndall, A.
AU - Czirják, L.
AU - Denton, C.
AU - Farge-Bancel, D.
AU - Kowal-Bielecka, O.
AU - Müller-Ladner, U.
AU - Bocelli-Tyndall, C.
AU - Matucci-Cerinic, M.
AU - Riemekasten, Gabriela
AU - Brückner, Claudia
AU - Airó, Paolo
AU - Scarsi, Mirko
AU - Scorza, Raffaella
AU - Beretta, Lorenzo
AU - Cozzi, Franco
AU - Tiso, Francesco
AU - Vonk, M. C.
AU - Van Den Hoogen, F. H.J.
AU - Wigley, Fredrick M.
AU - Hummers, Laura
AU - Nevskaya, Tatjana
AU - Ananieva, Lidia
AU - Miniati, Irene
AU - Tartaglia, Nicoletta
AU - Lomater, Claudia
AU - Balbir-Gurman, Alexandra
AU - Braun-Moscovici, Yolanda
AU - Bambara, Lisa Maria
AU - Caramaschi, Paola
AU - Valentini, Gabriele
AU - Ruocco, Luigia
AU - Krieg, Thomas
AU - Hunzelmann, Nicolas
AU - Varjú, Cecília
AU - Carriera, Patricia E.
AU - Joven, Beatriz
AU - Iannone, Florenzo
AU - Lapadula, Giovanni
AU - Kahan, André
AU - Allanore, Yannick
AU - Gabrielli, Armando
AU - Imperatore, Michele
AU - Scheja, Agneta
AU - Wollheim, Frank
AU - Damjanov, Nemanja
AU - Ostojic, Predrag
AU - Saar, Petra
AU - Tarner, Ingo H.
AU - Kötter, Ina
AU - Bombardieri, Stefano
AU - Bazzichi, Laura
AU - Del Papa, Nicoletta
AU - Comina, Denise P.
AU - Lo Monaco, Andrea
AU - La Corte, Renato
AU - Hachulla, Eric
AU - Launay, David
AU - Distler, Oliver
AU - Ciurea, Adrian
AU - Sierakowski, Stanislaw
AU - Mitchell, Holly
AU - Silver, Richard M.
AU - Krasowska, Dorota
AU - Michalska-Jakubus, Malgorzata
AU - Tikly, Mohammed
AU - Aboo, Nazrana
AU - Worm, Margitta
AU - Klaus, Pascal
AU - Rovenský, Jozef
AU - Lukáčová, Olga
AU - Rozman, Blaz
AU - Sipek, Alenka
AU - Clemente-Coelho, Paulo
AU - Shoenfeld, Yehuda
AU - Langewitch, Pnina
AU - Da Silva José, A. P.
AU - Salvador, M. J.
AU - Kuhn, Annegret
AU - Erdmann, Gunilla
AU - Bečvář, Radim
AU - Friedl, Elke
AU - Graninger, Winfried
AU - Riccieri, Valeria
AU - Caporali, Roberto
AU - Montecucco, Carlomaurizio
AU - Vlachoyiannopoulos, P.
AU - Distler, Meike
AU - Reich, Kristian
AU - Majdan, Maria
AU - Wielosz, Ewa
AU - Rednic, Simona
AU - Van Laar, Jacob M.
AU - Heitmann, Stefan
AU - Bruckner, Andreas
AU - Himsel, Andrea
AU - Riemann, Julia
AU - Meyringer, Rotraud
AU - Müller, Adelheid
AU - Martinovic, Duska
AU - Radic, Mislav
AU - Sticherling, Michael
AU - Szekanecz, Zoltan
AU - Szücs, Gabriella
AU - Giacomelli, Roberto
AU - Marrelli, Alessandra
AU - Stamenkovic, Bojana
AU - Stankovic, Aleksandra
AU - Aringer, Martin
AU - Smolen, Josef S.
AU - Kucharz, Eugene J.
AU - Kotulska, Anna T.
AU - Jablonska, Stefania
AU - Blasczik, Maria
AU - Jun, Jae Bum
AU - Mallia, Carmel
AU - Coleiro, Bernard
AU - Santamaria, Vera Ortiz
AU - Hinrichs, Ralf
AU - Nielsen, Henrik
AU - Cossutta, Roberta
AU - Ionescu, Ruxandra
AU - Opris, Daniela
AU - Steinbrink, Kerstin
AU - Grundt, Boris
AU - Bajocchi, Gianluigi
AU - Jiří, Štork
AU - Lefebvre, Paloma García De La Peña
AU - Zea Mendoza, Antonio C.
AU - Ribi, Camillo
AU - Chizzolini, Carlo
AU - Wisłowska, Margaret
AU - Novak, Srdan
AU - Indiveri, Francesco
AU - Jacobsen, Søren
AU - Frandsen, Per Brown
AU - Gorska, I. Zimmermann
AU - Tore Gran, Jane
AU - Midtvedt, Øyvind
AU - Ramos, Filipa Oliveira
AU - Rajcevska, Ljubinka Damjanovska
AU - Bozinovski, Georgi
AU - Schöffel, Dieter
AU - Sunderkötter, Cord
AU - Böhm, Markus
AU - Morović-Vergles, Jadranka
AU - Čulo, Melanie Ivana
AU - Cutolo, Maurizio
AU - Sulli, Alberto
AU - Derk, Chris T.
AU - Jimenez, Sergio A.
AU - Siakka, Panagiota
AU - Søndergaard, Klaus
AU - Stengaard-Pedersen, Kristian
AU - Cabane, Jean
AU - Tiev, Kiet Phong
AU - Mihai, Carina
AU - Sfrent-Cornateanu, Roxana
AU - Jendro, Michael
AU - Tuvik, Piia
AU - Antivalle, Marco
AU - Randisi, Giovanna
AU - Seidel, Matthias
AU - Clarenbach, Ricarda
AU - Simsek, Ismail
AU - Dinc, Ayhan
AU - Inanc, Murat
AU - Capraru, Monica Sinziana
AU - Capraru, Dorin
AU - Bañegil, Inmaculada
AU - Richter, Jutta
AU - Alhasani, Saad
AU - Földvari, Ivan
AU - Pinto, Sandra
AU - Brandão, Filipe
AU - Mas, Antonio Juan
PY - 2007/6
Y1 - 2007/6
N2 - Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.
AB - Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.
UR - http://www.scopus.com/inward/record.url?scp=34248379222&partnerID=8YFLogxK
U2 - 10.1136/ard.2006.062901
DO - 10.1136/ard.2006.062901
M3 - ???researchoutput.researchoutputtypes.contributiontojournal.article???
C2 - 17234652
AN - SCOPUS:34248379222
VL - 66
SP - 754
EP - 763
JO - Annals of the Rheumatic Diseases
JF - Annals of the Rheumatic Diseases
SN - 0003-4967
IS - 6
ER -