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Article Dans Une Revue Rheumatology Année : 2021

The clinical phenotype of systemic sclerosis patients with anti-pm/scl antibodies: results from the eustar cohort

Maria-Grazia Lazzaroni
  • Fonction : Auteur
Emiliano Marasco
  • Fonction : Auteur
Corrado Campochiaro
  • Fonction : Auteur
Jeska K. de Vries-Bouwstra
  • Fonction : Auteur
Jorge Rojas-Serrano
  • Fonction : Auteur
Elisabetta Zanatta
  • Fonction : Auteur
Simone Barsotti
  • Fonction : Auteur
Federica Furini
  • Fonction : Auteur
Konstantinos Triantafyllias
  • Fonction : Auteur
Giuseppina Abignano
  • Fonction : Auteur
Giacomo de Luca
  • Fonction : Auteur
Ellen de Langhe
  • Fonction : Auteur
Roger Hesselstrand
  • Fonction : Auteur
Francesca Ingegnoli
  • Fonction : Auteur
Eugenia Bertoldo
  • Fonction : Auteur
Vanessa Smith
  • Fonction : Auteur
Silvia Bellando-Randone
  • Fonction : Auteur
Hadi Poormoghim
  • Fonction : Auteur
Enrico Colombo
  • Fonction : Auteur
Angela Ceribelli
  • Fonction : Auteur
Alessio Furloni
  • Fonction : Auteur
Stefania Zingarelli
  • Fonction : Auteur
Ilaria Cavazzana
  • Fonction : Auteur
Franco Franceschini
  • Fonction : Auteur
Francesco del Galdo
  • Fonction : Auteur
Christopher P. Denton
  • Fonction : Auteur
Lorenzo Cavagna
  • Fonction : Auteur
Oliver Distler
  • Fonction : Auteur
Paolo Airo
  • Fonction : Auteur

Résumé

OBJECTIVE: To evaluate clinical associations of anti-PM/Scl antibodies in patients with Systemic Sclerosis (SSc) in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (SRC), malignancies, and functional outcome of interstitial lung disease (ILD). METHODS: (1) Analysis of SSc patients from the EUSTAR database: 144 anti-PM/Scl+ without SSc-specific autoantibodies were compared to 7,202 anti-PM/Scl-, and then to 155 anti-Pm/Scl+ with SSc-specific antibodies. (2) Case-control study: additional data were collected for 165 anti-PM/Scl+ SSc (85 from the EUSTAR registry), and compared to 257 anti-PM/Scl- SSc controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset. RESULTS: Patients with isolated anti-PM/Scl positivity, as compared with anti-Pm/Scl-, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of dermatomyositis, but similar frequency of SRC and malignancies (either synchronous with SSc onset or not). The presence of muscle involvement was associated with a more severe disease phenotype. Although very frequent, ILD had a better functional outcome in cases than in controls.In patients with both anti-PM/Scl and SSc-specific antibodies, a higher frequency of typical SSc features than in those with isolated anti-PM/Scl was observed. CONCLUSIONS: The analysis of the largest series of anti-PM/Scl+ SSc patients so far reported helps to delineate a specific clinical subset with muscle involvement, cutaneous dermatomyositis, calcinosis, and ILD characterized by a good functional outcome. SRC and malignancies do not seem to be part of this syndrome.

Dates et versions

hal-04490087 , version 1 (22-03-2024)

Identifiants

Citer

Maria-Grazia Lazzaroni, Emiliano Marasco, Corrado Campochiaro, Jeska K. de Vries-Bouwstra, Montserrat-Ixchel Gonzalez-Perez, et al.. The clinical phenotype of systemic sclerosis patients with anti-pm/scl antibodies: results from the eustar cohort. Rheumatology, 2021, Rheumatology, ⟨10.1093/rheumatology/keab152⟩. ⟨hal-04490087⟩
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