Title :

Late Skin Fibrosis in Systemic Sclerosis: A Study from the EUSTAR Cohort.

Author(s) :

Hughes, M. [Auteur]
Huang, S. [Auteur]
Alegre-Sancho, J. J. [Auteur]
Carreira, P. E. [Auteur]
Engelhart, M. [Auteur]
Hachulla, Eric [Auteur]
Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France [CeRAINO]
Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286
Henes, J. [Auteur]
Kerzberg, E. [Auteur]
Pozzi, M. R. [Auteur]
Riemekasten, G. [Auteur]
Smith, V. [Auteur]
Szücs, G. [Auteur]
Vanthuyne, M. [Auteur]
Zanatta, E. [Auteur]
Distler, O. [Auteur]
Gabrielli, A. G. [Auteur]
Hoffmann-Vold, A. M. [Auteur]
Steen, V. D. [Auteur]
Khanna, D. [Auteur]

Journal title :

Rheumatology

Abbreviated title :

Rheumatology (Oxford)

Volume number :

62

Pages :

SI54–SI63

Publication date :

2022-06-24

ISSN :

1462-0332

English keyword(s) :

SSc
scleroderma
skin
fibrosis
late disease
clinical trial design
cohort enrichment

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Objectives The early trajectory of skin fibrosis provides insights into the disease course of systemic sclerosis (SSc) including mortality; however, little is known about late skin fibrosis. The aims of our study were ...
Show more >Objectives The early trajectory of skin fibrosis provides insights into the disease course of systemic sclerosis (SSc) including mortality; however, little is known about late skin fibrosis. The aims of our study were to ascertain the prevalence and characteristics of late skin fibrosis in SSc. Methods We developed and tested three conceptual scenarios of late (>5 years after first non-RP feature) skin fibrosis including new worsening of skin disease, and failure to improve after worsening within 5-year window. We defined skin worsening as change in modified Rodnan skin score (mRSS) ≥5 units or ≥25%. Using strict inclusion criteria including complete mRSS, we identified 1,043 (out of 19 115) patients within the EUSTAR database for our analysis. We further restricted analysis within 887 (out of 1043) patients who had lcSSc or dcSSc at baseline. Results One-fifth of patients among the whole cohort (n = 208/1043, 19.9%) experienced mRSS worsening, including in patients with lcSSc or dcSSc at baseline (n = 193/887, 21.8%). This was largely due to new skin worsening after the 5-year window or failure to improve with worsening within the 5-year window. Patients with lower baseline mRSS and lcSSc were more likely to develop late skin fibrosis. Anti-Scl-70 was associated with progression from baseline lcSSc to dcSSc, and anticentromere was protective. Conclusions Late skin fibrosis is not uncommon in SSc. We have identified different patterns relevant to clinical practice and trial design. Late skin fibrosis is a neglected manifestation of SSc and warrants further investigation including to determine clinical outcomes and optimal therapeutic strategy.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

Université de Lille
Inserm
CHU Lille

Collections :

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

Submission date :

2024-01-12T01:12:54Z
2024-03-15T10:14:33Z