Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

Carina Mihai; Oliver Distler; Ana Maria Gheorghiu; Paul I. Constantin; Rucsandra Dobrota; Suzana Jordan; Vanessa Smith; Eric Hachulla; Jörg Henes; Elise Siegert; Serena Vettori; Ulf Müller-Ladner; Marco Matucci Cerinic; Yannick Allanore; Gemma Lepri; Veronika K. Jaeger; Ulrich A. Walker; Florenzo Iannone; Fabio Cacciapaglia; Michal Tomčík; Radim Becvar; Simona Rednic; Ana Petcu; Iulia Szabo; Veronica Codullo; Roberto Caporali; Carlomaurizio Montecucco; Patricia Carreira; Beatriz Ioven; Tunde Minier; László Czirják; Carlo Chizzolini; Danièle Allali; Elisabetta Zanatta; Andrea Doria; Armando Gabrielli; Paolo Airò; Maria Grazia Lazzaroni; Mislav Radić; Duska Martinovic; Yolanda Braun-Moscovici; Alexandra Balbir-Gurman; Nicolas Hunzelmann; Paola Caramaschi; Jadranka Morovic-Vergles; Christopher Denton; Vera Ortiz Santamaria; Stefan Heitmann; Dorota Krasowska; Malgorzata Michalska-Jakubus; Matthias Seidel; Ivan Foeldvari; Nicola Helmus; Maria João Salvador; Bojana Stamenkovic; Aleksandra Stankovic; Lidia Ananieva; Ariane Herrick; Merete Engelhart; Carlos De La Puente; Anna Maria Hoffmann-Vold; Øyvind Midtvedt; David Launay; Vincent Sobanski; Valeria Riccieri; Daniela Opris-Belinski; Laura Groseanu; Ruxandra Maria Ionescu; Mihai Bojinca; Cord Sunderkötter; Jörg Distler; Francesca Ingegnoli; Amber Van Der Haecke; Susanne Ullman; Maria Rosa Pozzi; Kilian Eyerich; Marie Vanthuyne; Anne Erler; Martin Aringer; Ellen De Langhe; Marko Baresic; Miroslav Mayer; Branimir Anic; Sule Yavuz; Brigitte Granel; Sergei Popa; Svetlana Agachi; Thierry Zenone; Alessandro Mathieu; Alessandra Vacca; Kamal Solanki; Douglas Veale; Esthela Loyo; Carmen Tineo; Antonetta Gigante; Edoardo Rosato; Fahrettin Oksel; Figen Yagurcu; Cristina Mihaela Tǎnǎseanu; Elisa Visalli; Alessia Benenati; Rosario Foti; Codrina Ancuta; Diana Dan; Sabine Adler; Peter Villiger; Nihal Fathi; Paloma García De La Peña Lefebvre; Jorge Juan González Martín; Emmanuel Chatelus; Jean Sibilia; Ira Litinsky; Francesco Del Galdo; Lesley Ann Sakettkoo; Eduardo Kerzberg; Washington A. Bianchi; Breno Valdetaro Bianchi; Ivan Castellví; Massimiliano Limonta; Doron Rimar; Maura Couto; Camillo Ribi; François Spertini; Sarah Kahl; Vivien Hsu; Vincent Poindron; Kilifa Meghit; Thierry Martin; Kathleen Kolstad; Lorinda Chung; Astrid Thiele; Tim Schmeiser; Zbigniew Zdrojewski; Gabriela Riemekasten; Yair Levy; Anca Cardoneanu; Alexandra Burlui; Elena Rezus; Omer Nuri Pamuk; Rossella Talotta; Sara Bongiovanni; Piercarlo Sarzi Puttini

doi:10.1093/rheumatology/kez558

Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

Carina Mihai, Oliver Distler, Ana Maria Gheorghiu, Paul I. Constantin, Rucsandra Dobrota, Suzana Jordan, Vanessa Smith, Eric Hachulla, Jörg Henes, Elise Siegert, Serena Vettori, Ulf Müller-Ladner, Marco Matucci Cerinic, Yannick Allanore, Gemma Lepri, Veronika K. Jaeger, Ulrich A. Walker, Florenzo Iannone, Fabio Cacciapaglia, Michal TomčíkRadim Becvar, Simona Rednic, Ana Petcu, Iulia Szabo, Veronica Codullo, Roberto Caporali, Carlomaurizio Montecucco, Patricia Carreira, Beatriz Ioven, Tunde Minier, László Czirják, Carlo Chizzolini, Danièle Allali, Elisabetta Zanatta, Andrea Doria, Armando Gabrielli, Paolo Airò, Maria Grazia Lazzaroni, Mislav Radić, Duska Martinovic, Yolanda Braun-Moscovici, Alexandra Balbir-Gurman, Nicolas Hunzelmann, Paola Caramaschi, Jadranka Morovic-Vergles, Christopher Denton, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Malgorzata Michalska-Jakubus, Matthias Seidel, Ivan Foeldvari, Nicola Helmus, Maria João Salvador, Bojana Stamenkovic, Aleksandra Stankovic, Lidia Ananieva, Ariane Herrick, Merete Engelhart, Carlos De La Puente, Anna Maria Hoffmann-Vold, Øyvind Midtvedt, David Launay, Vincent Sobanski, Valeria Riccieri, Daniela Opris-Belinski, Laura Groseanu, Ruxandra Maria Ionescu, Mihai Bojinca, Cord Sunderkötter, Jörg Distler, Francesca Ingegnoli, Amber Van Der Haecke, Susanne Ullman, Maria Rosa Pozzi, Kilian Eyerich, Marie Vanthuyne, Anne Erler, Martin Aringer, Ellen De Langhe, Marko Baresic, Miroslav Mayer, Branimir Anic, Sule Yavuz, Brigitte Granel, Sergei Popa, Svetlana Agachi, Thierry Zenone, Alessandro Mathieu, Alessandra Vacca, Kamal Solanki, Douglas Veale, Esthela Loyo, Carmen Tineo, Antonetta Gigante, Edoardo Rosato, Fahrettin Oksel, Figen Yagurcu, Cristina Mihaela Tǎnǎseanu, Elisa Visalli, Alessia Benenati, Rosario Foti, Codrina Ancuta, Diana Dan, Sabine Adler, Peter Villiger, Nihal Fathi, Paloma García De La Peña Lefebvre, Jorge Juan González Martín, Emmanuel Chatelus, Jean Sibilia, Ira Litinsky, Francesco Del Galdo, Lesley Ann Sakettkoo, Eduardo Kerzberg, Washington A. Bianchi, Breno Valdetaro Bianchi, Ivan Castellví, Massimiliano Limonta, Doron Rimar, Maura Couto, Camillo Ribi, François Spertini, Sarah Kahl, Vivien Hsu, Vincent Poindron, Kilifa Meghit, Thierry Martin, Kathleen Kolstad, Lorinda Chung, Astrid Thiele, Tim Schmeiser, Zbigniew Zdrojewski, Gabriela Riemekasten, Yair Levy, Anca Cardoneanu, Alexandra Burlui, Elena Rezus, Omer Nuri Pamuk, Rossella Talotta, Sara Bongiovanni, Piercarlo Sarzi Puttini

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Abstract

Objective: In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods: We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results: 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion: In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.

Original language	English
Pages (from-to)	2016-2023
Number of pages	8
Journal	Rheumatology
Volume	59
Issue number	8
DOIs	https://doi.org/10.1093/rheumatology/kez558
State	Published - 1 Aug 2020

Keywords

digital ulcers
gangrene
systemic sclerosis
vasculopathy

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10.1093/rheumatology/kez558

Cite this

Mihai, C., Distler, O., Gheorghiu, A. M., Constantin, P. I., Dobrota, R., Jordan, S., Smith, V., Hachulla, E., Henes, J., Siegert, E., Vettori, S., Müller-Ladner, U., Matucci Cerinic, M., Allanore, Y., Lepri, G., Jaeger, V. K., Walker, U. A., Iannone, F., Cacciapaglia, F., ... Puttini, P. S. (2020). Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort. Rheumatology, 59(8), 2016-2023. https://doi.org/10.1093/rheumatology/kez558

@article{ca1e35ef5a064efbbc8028e05bd8c6a7,

title = "Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort",

abstract = "Objective: In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods: We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results: 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion: In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.",

keywords = "digital ulcers, gangrene, systemic sclerosis, vasculopathy",

author = "Carina Mihai and Oliver Distler and Gheorghiu, {Ana Maria} and Constantin, {Paul I.} and Rucsandra Dobrota and Suzana Jordan and Vanessa Smith and Eric Hachulla and J{\"o}rg Henes and Elise Siegert and Serena Vettori and Ulf M{\"u}ller-Ladner and {Matucci Cerinic}, Marco and Yannick Allanore and Gemma Lepri and Jaeger, {Veronika K.} and Walker, {Ulrich A.} and Florenzo Iannone and Fabio Cacciapaglia and Michal Tom{\v c}{\'i}k and Radim Becvar and Simona Rednic and Ana Petcu and Iulia Szabo and Veronica Codullo and Roberto Caporali and Carlomaurizio Montecucco and Patricia Carreira and Beatriz Ioven and Tunde Minier and L{\'a}szl{\'o} Czirj{\'a}k and Carlo Chizzolini and Dani{\`e}le Allali and Elisabetta Zanatta and Andrea Doria and Armando Gabrielli and Paolo Air{\`o} and Lazzaroni, {Maria Grazia} and Mislav Radi{\'c} and Duska Martinovic and Yolanda Braun-Moscovici and Alexandra Balbir-Gurman and Nicolas Hunzelmann and Paola Caramaschi and Jadranka Morovic-Vergles and Christopher Denton and Santamaria, {Vera Ortiz} and Stefan Heitmann and Dorota Krasowska and Malgorzata Michalska-Jakubus and Matthias Seidel and Ivan Foeldvari and Nicola Helmus and Salvador, {Maria Jo{\~a}o} and Bojana Stamenkovic and Aleksandra Stankovic and Lidia Ananieva and Ariane Herrick and Merete Engelhart and {De La Puente}, Carlos and Hoffmann-Vold, {Anna Maria} and {\O}yvind Midtvedt and David Launay and Vincent Sobanski and Valeria Riccieri and Daniela Opris-Belinski and Laura Groseanu and Ionescu, {Ruxandra Maria} and Mihai Bojinca and Cord Sunderk{\"o}tter and J{\"o}rg Distler and Francesca Ingegnoli and {Van Der Haecke}, Amber and Susanne Ullman and Pozzi, {Maria Rosa} and Kilian Eyerich and Marie Vanthuyne and Anne Erler and Martin Aringer and {De Langhe}, Ellen and Marko Baresic and Miroslav Mayer and Branimir Anic and Sule Yavuz and Brigitte Granel and Sergei Popa and Svetlana Agachi and Thierry Zenone and Alessandro Mathieu and Alessandra Vacca and Kamal Solanki and Douglas Veale and Esthela Loyo and Carmen Tineo and Antonetta Gigante and Edoardo Rosato and Fahrettin Oksel and Figen Yagurcu and Tǎnǎseanu, {Cristina Mihaela} and Elisa Visalli and Alessia Benenati and Rosario Foti and Codrina Ancuta and Diana Dan and Sabine Adler and Peter Villiger and Nihal Fathi and {De La Pe{\~n}a Lefebvre}, {Paloma Garc{\'i}a} and Mart{\'i}n, {Jorge Juan Gonz{\'a}lez} and Emmanuel Chatelus and Jean Sibilia and Ira Litinsky and {Del Galdo}, Francesco and Sakettkoo, {Lesley Ann} and Eduardo Kerzberg and Bianchi, {Washington A.} and Bianchi, {Breno Valdetaro} and Ivan Castellv{\'i} and Massimiliano Limonta and Doron Rimar and Maura Couto and Camillo Ribi and Fran{\c c}ois Spertini and Sarah Kahl and Vivien Hsu and Vincent Poindron and Kilifa Meghit and Thierry Martin and Kathleen Kolstad and Lorinda Chung and Astrid Thiele and Tim Schmeiser and Zbigniew Zdrojewski and Gabriela Riemekasten and Yair Levy and Anca Cardoneanu and Alexandra Burlui and Elena Rezus and Pamuk, {Omer Nuri} and Rossella Talotta and Sara Bongiovanni and Puttini, {Piercarlo Sarzi}",

note = "Publisher Copyright: {\textcopyright} 2019 The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].",

year = "2020",

month = aug,

day = "1",

doi = "10.1093/rheumatology/kez558",

language = "English",

volume = "59",

pages = "2016--2023",

journal = "Rheumatology",

issn = "1462-0324",

publisher = "Oxford University Press",

number = "8",

}

Mihai, C, Distler, O, Gheorghiu, AM, Constantin, PI, Dobrota, R, Jordan, S, Smith, V, Hachulla, E, Henes, J, Siegert, E, Vettori, S, Müller-Ladner, U, Matucci Cerinic, M, Allanore, Y, Lepri, G, Jaeger, VK, Walker, UA, Iannone, F, Cacciapaglia, F, Tomčík, M, Becvar, R, Rednic, S, Petcu, A, Szabo, I, Codullo, V, Caporali, R, Montecucco, C, Carreira, P, Ioven, B, Minier, T, Czirják, L, Chizzolini, C, Allali, D, Zanatta, E, Doria, A, Gabrielli, A, Airò, P, Lazzaroni, MG, Radić, M, Martinovic, D, Braun-Moscovici, Y, Balbir-Gurman, A, Hunzelmann, N, Caramaschi, P, Morovic-Vergles, J, Denton, C, Santamaria, VO, Heitmann, S, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Foeldvari, I, Helmus, N, Salvador, MJ, Stamenkovic, B, Stankovic, A, Ananieva, L, Herrick, A, Engelhart, M, De La Puente, C, Hoffmann-Vold, AM, Midtvedt, Ø, Launay, D, Sobanski, V, Riccieri, V, Opris-Belinski, D, Groseanu, L, Ionescu, RM, Bojinca, M, Sunderkötter, C, Distler, J, Ingegnoli, F, Van Der Haecke, A, Ullman, S, Pozzi, MR, Eyerich, K, Vanthuyne, M, Erler, A, Aringer, M, De Langhe, E, Baresic, M, Mayer, M, Anic, B, Yavuz, S, Granel, B, Popa, S, Agachi, S, Zenone, T, Mathieu, A, Vacca, A, Solanki, K, Veale, D, Loyo, E, Tineo, C, Gigante, A, Rosato, E, Oksel, F, Yagurcu, F, Tǎnǎseanu, CM, Visalli, E, Benenati, A, Foti, R, Ancuta, C, Dan, D, Adler, S, Villiger, P, Fathi, N, De La Peña Lefebvre, PG, Martín, JJG, Chatelus, E, Sibilia, J, Litinsky, I, Del Galdo, F, Sakettkoo, LA, Kerzberg, E, Bianchi, WA, Bianchi, BV, Castellví, I, Limonta, M, Rimar, D, Couto, M, Ribi, C, Spertini, F, Kahl, S, Hsu, V, Poindron, V, Meghit, K, Martin, T, Kolstad, K, Chung, L, Thiele, A, Schmeiser, T, Zdrojewski, Z, Riemekasten, G, Levy, Y, Cardoneanu, A, Burlui, A, Rezus, E, Pamuk, ON, Talotta, R, Bongiovanni, S & Puttini, PS 2020, 'Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort', Rheumatology, vol. 59, no. 8, pp. 2016-2023. https://doi.org/10.1093/rheumatology/kez558

TY - JOUR

T1 - Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

AU - Mihai, Carina

AU - Distler, Oliver

AU - Gheorghiu, Ana Maria

AU - Constantin, Paul I.

AU - Dobrota, Rucsandra

AU - Jordan, Suzana

AU - Smith, Vanessa

AU - Hachulla, Eric

AU - Henes, Jörg

AU - Siegert, Elise

AU - Vettori, Serena

AU - Müller-Ladner, Ulf

AU - Matucci Cerinic, Marco

AU - Allanore, Yannick

AU - Lepri, Gemma

AU - Jaeger, Veronika K.

AU - Walker, Ulrich A.

AU - Iannone, Florenzo

AU - Cacciapaglia, Fabio

AU - Tomčík, Michal

AU - Becvar, Radim

AU - Rednic, Simona

AU - Petcu, Ana

AU - Szabo, Iulia

AU - Codullo, Veronica

AU - Caporali, Roberto

AU - Montecucco, Carlomaurizio

AU - Carreira, Patricia

AU - Ioven, Beatriz

AU - Minier, Tunde

AU - Czirják, László

AU - Chizzolini, Carlo

AU - Allali, Danièle

AU - Zanatta, Elisabetta

AU - Doria, Andrea

AU - Gabrielli, Armando

AU - Airò, Paolo

AU - Lazzaroni, Maria Grazia

AU - Radić, Mislav

AU - Martinovic, Duska

AU - Braun-Moscovici, Yolanda

AU - Balbir-Gurman, Alexandra

AU - Hunzelmann, Nicolas

AU - Caramaschi, Paola

AU - Morovic-Vergles, Jadranka

AU - Denton, Christopher

AU - Santamaria, Vera Ortiz

AU - Heitmann, Stefan

AU - Krasowska, Dorota

AU - Michalska-Jakubus, Malgorzata

AU - Seidel, Matthias

AU - Foeldvari, Ivan

AU - Helmus, Nicola

AU - Salvador, Maria João

AU - Stamenkovic, Bojana

AU - Stankovic, Aleksandra

AU - Ananieva, Lidia

AU - Herrick, Ariane

AU - Engelhart, Merete

AU - De La Puente, Carlos

AU - Hoffmann-Vold, Anna Maria

AU - Midtvedt, Øyvind

AU - Launay, David

AU - Sobanski, Vincent

AU - Riccieri, Valeria

AU - Opris-Belinski, Daniela

AU - Groseanu, Laura

AU - Ionescu, Ruxandra Maria

AU - Bojinca, Mihai

AU - Sunderkötter, Cord

AU - Distler, Jörg

AU - Ingegnoli, Francesca

AU - Van Der Haecke, Amber

AU - Ullman, Susanne

AU - Pozzi, Maria Rosa

AU - Eyerich, Kilian

AU - Vanthuyne, Marie

AU - Erler, Anne

AU - Aringer, Martin

AU - De Langhe, Ellen

AU - Baresic, Marko

AU - Mayer, Miroslav

AU - Anic, Branimir

AU - Yavuz, Sule

AU - Granel, Brigitte

AU - Popa, Sergei

AU - Agachi, Svetlana

AU - Zenone, Thierry

AU - Mathieu, Alessandro

AU - Vacca, Alessandra

AU - Solanki, Kamal

AU - Veale, Douglas

AU - Loyo, Esthela

AU - Tineo, Carmen

AU - Gigante, Antonetta

AU - Rosato, Edoardo

AU - Oksel, Fahrettin

AU - Yagurcu, Figen

AU - Tǎnǎseanu, Cristina Mihaela

AU - Visalli, Elisa

AU - Benenati, Alessia

AU - Foti, Rosario

AU - Ancuta, Codrina

AU - Dan, Diana

AU - Adler, Sabine

AU - Villiger, Peter

AU - Fathi, Nihal

AU - De La Peña Lefebvre, Paloma García

AU - Martín, Jorge Juan González

AU - Chatelus, Emmanuel

AU - Sibilia, Jean

AU - Litinsky, Ira

AU - Del Galdo, Francesco

AU - Sakettkoo, Lesley Ann

AU - Kerzberg, Eduardo

AU - Bianchi, Washington A.

AU - Bianchi, Breno Valdetaro

AU - Castellví, Ivan

AU - Limonta, Massimiliano

AU - Rimar, Doron

AU - Couto, Maura

AU - Ribi, Camillo

AU - Spertini, François

AU - Kahl, Sarah

AU - Hsu, Vivien

AU - Poindron, Vincent

AU - Meghit, Kilifa

AU - Martin, Thierry

AU - Kolstad, Kathleen

AU - Chung, Lorinda

AU - Thiele, Astrid

AU - Schmeiser, Tim

AU - Zdrojewski, Zbigniew

AU - Riemekasten, Gabriela

AU - Levy, Yair

AU - Cardoneanu, Anca

AU - Burlui, Alexandra

AU - Rezus, Elena

AU - Pamuk, Omer Nuri

AU - Talotta, Rossella

AU - Bongiovanni, Sara

AU - Puttini, Piercarlo Sarzi

N1 - Publisher Copyright: © 2019 The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].

PY - 2020/8/1

Y1 - 2020/8/1

N2 - Objective: In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods: We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results: 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion: In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.

AB - Objective: In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods: We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results: 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion: In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.

KW - digital ulcers

KW - gangrene

KW - systemic sclerosis

KW - vasculopathy

UR - http://www.scopus.com/inward/record.url?scp=85080054194&partnerID=8YFLogxK

U2 - 10.1093/rheumatology/kez558

DO - 10.1093/rheumatology/kez558

M3 - Article

C2 - 31790136

AN - SCOPUS:85080054194

SN - 1462-0324

VL - 59

SP - 2016

EP - 2023

JO - Rheumatology

JF - Rheumatology

IS - 8

ER -

Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

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