TY - JOUR
T1 - Does regression of skin thickening predict improvement of internal organ involvement and survival in patients with diffuse cutaneous systemic sclerosis? A EUSTAR analysis
AU - EUSTAR Collaborators
AU - Wyss, Anja
AU - Jordan, Suzana
AU - Graf, Nicole
AU - Carreira, Patricia E.
AU - Distler, Jörg
AU - Cerinic, Marco Matucci
AU - Siegert, Elise
AU - Henes, Jörg
AU - Zanatta, Elisabetta
AU - Riccieri, Valeria
AU - Truchetet, Marie Elise
AU - Oksel, Fahrettin
AU - Li, Mengtao
AU - Kucharz, Eugene J.
AU - Eyerich, Kilian
AU - Del Galdo, Francesco
AU - Vonk, Madelon C.
AU - Vold, Anna Maria Hoffman
AU - Gabrielli, Armando
AU - Distler, Oliver
AU - Dagna, Lorenzo
AU - de Vries-Bouwstra, Jeska
AU - Ramazan, Ana Maria
AU - Furst, Daniel
AU - Sfikakis, Petros
AU - Kötter, Ina
AU - Sarzi Puttini, Piercarlo
AU - Rezus, Elena
AU - Riemekasten, Gabriela
AU - Bernardino, Vera
AU - Majewski, Dominik
AU - Schmeiser, Tim
AU - Chung, Lorinda S.
AU - Martin, Thierry
AU - Spertini, François
AU - Limonta, Massimiliano
AU - Kerzberg, Eduardo
AU - Saketkoo, Lesley Ann
AU - Seskute, Goda
AU - Del Galdo, Francesco
AU - Litinsky, Ira
AU - Sibilia, Jean
AU - dela Peña Lefebvre, Paloma García
AU - Maurer, Britta
AU - Ancuta, Codrina
AU - Foti, Rosario
AU - Tanaseanu, Cristina Mihaela
AU - Loyo, Esthela
AU - Veale, Douglas
AU - Solanki, Kamal
N1 - Publisher Copyright:
© The Author(s) 2024.
PY - 2024/12
Y1 - 2024/12
N2 - Objective: Patients with diffuse cutaneous systemic sclerosis (dcSSc) frequently show spontaneous improvement of skin fibrosis. Our aim was to examine whether an improvement in skin fibrosis predicts lower likelihood of visceral organ progression and better survival. Methods: Patients from the European Scleroderma Trials and Research (EUSTAR) cohort with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, and valid mRSS at 12±3 months follow up were included. Regression/progression of skin fibrosis was defined as a decrease/increase in mRSS >5 points and≥25% from baseline to follow up. The outcomes included progression of lung, renal, cardiac and gastrointestinal manifestations using consensus derived definitions and all-cause death. Regressive, stable and progressive patients were compared by univariate, Kaplan-Meier survival curve and Cox regression analysis. Results: Of 1257 included patients, 883 (70.2%) were stable, 282 (22.4%) regressive, and 92 (7.3%) progressive. Regressive patients, adjusted for baseline mRSS, baseline immunosuppression, baseline FVC, and disease duration, showed a significantly lower probability of FVC decline ≥10% than progressive patients (p=0.00003), lower probability of all-cause mortality during follow up (p=0.035) compared to progressive patients.Improvement of skin fibrosis was not associated with progression of other organ manifestations. Conclusion: We found that regression of skin fibrosis is associated with a lower probability of lung progression and better survival at follow up. The link between the disease course of skin and lung fibrosis in SSc can help to better stratify patients in clinical practice and enrich for ILD progressive patients in clinical trials.
AB - Objective: Patients with diffuse cutaneous systemic sclerosis (dcSSc) frequently show spontaneous improvement of skin fibrosis. Our aim was to examine whether an improvement in skin fibrosis predicts lower likelihood of visceral organ progression and better survival. Methods: Patients from the European Scleroderma Trials and Research (EUSTAR) cohort with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, and valid mRSS at 12±3 months follow up were included. Regression/progression of skin fibrosis was defined as a decrease/increase in mRSS >5 points and≥25% from baseline to follow up. The outcomes included progression of lung, renal, cardiac and gastrointestinal manifestations using consensus derived definitions and all-cause death. Regressive, stable and progressive patients were compared by univariate, Kaplan-Meier survival curve and Cox regression analysis. Results: Of 1257 included patients, 883 (70.2%) were stable, 282 (22.4%) regressive, and 92 (7.3%) progressive. Regressive patients, adjusted for baseline mRSS, baseline immunosuppression, baseline FVC, and disease duration, showed a significantly lower probability of FVC decline ≥10% than progressive patients (p=0.00003), lower probability of all-cause mortality during follow up (p=0.035) compared to progressive patients.Improvement of skin fibrosis was not associated with progression of other organ manifestations. Conclusion: We found that regression of skin fibrosis is associated with a lower probability of lung progression and better survival at follow up. The link between the disease course of skin and lung fibrosis in SSc can help to better stratify patients in clinical practice and enrich for ILD progressive patients in clinical trials.
KW - Observation studies
KW - Rare diseases
KW - Respiratory
KW - Scleroderma and related disorders
KW - Skin
UR - http://www.scopus.com/inward/record.url?scp=85208291602&partnerID=8YFLogxK
U2 - 10.1186/s13075-024-03418-2
DO - 10.1186/s13075-024-03418-2
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C2 - 39482761
AN - SCOPUS:85208291602
SN - 1478-6354
VL - 26
JO - Arthritis Research and Therapy
JF - Arthritis Research and Therapy
IS - 1
M1 - 187
ER -