Title :

Outcomes of limited cutaneous systemic sclerosis patients: results on more than 12,000 patients from the eustar database

Author(s) :

Frantz, Camelia [Auteur]
Université Paris Descartes - Paris 5 [UPD5]
Huscher, Dorte [Auteur]
Avouac, Jerome [Auteur]
Université Paris Descartes - Paris 5 [UPD5]
Hachulla, Eric [Auteur]
Lille Inflammation Research International Center - U 995 [LIRIC]
Balbir-Gurman, Alexandra [Auteur]
Riemekasten, Gabriela [Auteur]
Siegert, Elise [Auteur]
Lazzaroni, Maria-Grazia [Auteur]
Carreira, Patricia E. [Auteur]
Vettori, Serena [Auteur]
Zanatta, Elisabetta [Auteur]
Ullman, Susanne [Auteur]
Czirjak, Laszlo [Auteur]
Kowal-Bielecka, Otylia [Auteur]
Distler, Oliver [Auteur]
Matucci-Cerinic, Marco [Auteur]
Allanore, Yannick [Auteur]
Université Paris Descartes - Paris 5 [UPD5]

Journal title :

Autoimmunity Reviews

Abbreviated title :

Autoimmun Rev

Volume number :

19

Pages :

102452

Publication date :

2020-02

ISSN :

1873-0183

English keyword(s) :

Fibrosis
Interstitial lung disease
Digital ulcers
Limited cutaneous systemic sclerosis
Outcomes

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

OBJECTIVE: Limited cutaneous systemic sclerosis (LcSSc) is the most common subset of SSc but it has been overlooked in the past years. At a time at which clinical trials focus on diffuse cutaneous SSc (DcSSc) we aimed at ...
Show more >OBJECTIVE: Limited cutaneous systemic sclerosis (LcSSc) is the most common subset of SSc but it has been overlooked in the past years. At a time at which clinical trials focus on diffuse cutaneous SSc (DcSSc) we aimed at clarifying the outcomes of LcSSc and at evaluating whether potential drug positioned in DcSSc may also be used in LcSSc. METHODS: The EUSTAR database was used to investigate skin, lung and peripheral vasculopathy outcomes in LcSSc. Worsening of skin fibrosis, ILD and peripheral vasculopathy were defined by an increase in modified Rodnan skin score (mRSS) > 3.5 points, a decrease of FVC > 10% in patients with ILD at baseline, and by the development of new digital ulcers (DU) in patients without DU at baseline. RESULTS: 8013 LcSSc and 4786 DcSSc patients were included. In contrast to DcSSc, skin disease was remarkably stable in the majority of LcSSc patients with >80% having a change lower than ±4 units of mRSS at 12, 24 and 36 months follow-up. Conversely, FVC changes over time were very similar between LcSSc and DcSSc. Regarding DU, numbers of patients with new DU over time seemed to be almost similar between the two subsets. CONCLUSIONS: LcSSc patients have a low mRSS at baseline with marginal changes with time. Conversely, SSc-ILD can be as progressive as in DcSSc supporting the inclusion of LcSSc patients in SSc-ILD trials and suggesting potential benefit of any anti-ILD drugs. Similarly, although slightly less common, DU should receive the same attention in the two subsets.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

CHU Lille
Inserm
Université de Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-01-30T10:28:16Z
2024-04-25T12:46:41Z