Does regression of skin thickening predict improvement of internal organ involvement and survival in patients with diffuse cutaneous systemic sclerosis? A EUSTAR analysis

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2024年10月31日

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Anja Wyss,
Suzana Jordan,
Nicole Graf,
Patricia E. Carreira,
Jörg Distler,
Marco Matucci Cerinic,
Elise Siegert,
Jörg Henes,
Elisabetta Zanatta,
Valeria Riccieri,
Marie-Elise Truchetet,
Fahrettin Oksel,
Mengtao Li,
Eugene J. Kucharz,
Kilian Eyerich,
Francesco Del Galdo,
Madelon C. Vonk,
Anna-Maria Hoffman Vold,
Armando Gabrielli,
Oliver Distler,
Ulrich Walker,
Florenzo Iannone,
Radim Becvar,
Giovana Cuomo,
Simona Rednic,
Yannick Allanore,
C. Montecucco,
Srdan Novak,
László Czirják,
Michele Iudici,
Katja Perdan Pirkmajer,
Bernard Coleiro,
Dominique Farge Bancel,
Paolo Airò,
Roger Hesselstrand,
Mislav Radic,
Alexandra Balbir-Gurman,
Nicolas Hunzelmann,
Raffaele Pellerito,
Alessandro Giollo,
Christopher Denton,
Nemanja Damjanov,
Vera Ortiz-Santamaria,
Stefan Heitmann,
Matthias Seidel,
Maria João Salvador,
Bojana Stamenkovic,
Carlo Francesco Selmi,
Mohammed Tikly,
Lidia P. Ananieva,
Ulf Müller-Ladner,
Merete Engelhart,
Eric Hachulla,
Ruxandra Maria Ionescu,
Ana Maria Gheorghiu,
Cord Sunderkötter,
Francesca Ingegnoli,
Luc Mouthon,
Vanessa Smith,
Francesco Paolo Cantatore,
Susanne Ullman,
Maria Rosa Pozzi,
Piotr Wiland,
Juan Jose Alegre-Sancho,
Brigitte Krummel-Lorenz,
Kristine Herrmann,
Ellen De Langhe,
Branimir Anic,
Maria Üprus,
Sule Yavuz,
Carolina de Souza Müller,
Svetlana Agachi,
Thierry Zenone,
Simon Stebbings,
Alessandra Vacca,
Lisa Stamp,
Kamal Solanki,
Douglas Veale,
Esthela Loyo,
Cristina-Mihaela Tanaseanu,
Rosario Foti,
Codrina Ancuta,
Britta Maurer,
Paloma García dela Peña Lefebvre,
Jean Sibilia,
Ira Litinsky,
Francesco Del Galdo,
Goda Seskute,
Lesley Ann Saketkoo,
Eduardo Kerzberg,
Massimiliano Limonta,
François Spertini,
Thierry Martin,
Lorinda S Chung,
Tim Schmeiser,
Dominik Majewski,
Vera Bernardino,
Gabriela Riemekasten,
Elena Rezus,
Piercarlo Sarzi Puttini,
Ina Kötter,
Petros Sfikakis,
Daniel Furst,
Ana-Maria Ramazan,
Jeska de Vries-Bouwstra,
Lorenzo Dagna

摘要

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.