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. 2024 Oct 31;26(1):187.
doi: 10.1186/s13075-024-03418-2.

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

Anja Wyss  1 , Suzana Jordan  1 , Nicole Graf  2 , Patricia E Carreira  3 , Jörg Distler  4   5 , Marco Matucci Cerinic  6 , Elise Siegert  7 , Jörg Henes  8 , Elisabetta Zanatta  9 , Valeria Riccieri  10 , Marie-Elise Truchetet  11 , Fahrettin Oksel  12 , Mengtao Li  13 , Eugene J Kucharz  14 , Kilian Eyerich  15 , Francesco Del Galdo  16 , Madelon C Vonk  17 , Anna-Maria Hoffman Vold  18 , Armando Gabrielli  5   19 , Oliver Distler  20 ; EUSTAR collaborators
Collaborators, Affiliations

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

Anja Wyss et al. Arthritis Res Ther. .

Abstract

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.

Keywords: Observation studies; Rare diseases; Respiratory; Scleroderma and related disorders; Skin.

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Conflict of interest statement

None: AG, SJ; NG, PEC; JHD; MMC, ES, JH; VR, FO, ML,EJK, KE, FDG, MV; AMHV, AG;EZ: speaking and lecture fees, consulting/advisory: Janssen, GSK, Boehringer Ingelheim. MET has/had consultancy relationship with and/or has received research funding from and/or has served as a speaker for the following companies in the area of potential treatments for systemic sclerosis and its complications in the last three calendar years: Abbvie, Boehringer Ingelheim, Janssen, UCB, Lilly, Pfizer and Novartis.OD has/had consultancy relationship with and/or has received research funding from and/or has served as a speaker for the following companies in the area of potential treatments for systemic sclerosis and its complications in the last three calendar years: 4P-Pharma, Abbvie, Acceleron, Alcimed, Altavant, Amgen, AnaMar, Argenx, Arxx, AstraZeneca, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galderma, Galapagos, Glenmark, Gossamer, Horizon, Janssen, Kymera, Lupin, Medscape, Merck, Miltenyi Biotec, Mitsubishi Tanabe, Novartis, Orion, Prometheus, Redxpharma, Roivant, Topadur and UCB. Patent issued “mir-29 for the treatment of systemic sclerosis” (US8247389, EP2331143). Co-founder of CITUS AG.

Figures

Fig. 1
Fig. 1
Probability of lung function progression depending on previous change of skin fibrosis
Fig. 2
Fig. 2
Probability of intestinal progression depending on previous change of skin fibrosis
Fig. 3
Fig. 3
Probability of severe intestinal progression depending on previous change of skin fibrosis
Fig. 4
Fig. 4
Probability of all-cause death depending on previous change of skin fibrosis
See this image and copyright information in PMC

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