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The clinical phenotype of systemic sclerosis patients with anti-PM/Scl antibodies: results from the EUSTAR cohort

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@article{d5c423fb8bfa4270a6ce7dfa74b4df1e,
title = "The clinical phenotype of systemic sclerosis patients with anti-PM/Scl antibodies: results from the EUSTAR cohort",
abstract = "OBJECTIVE: To evaluate clinical associations of anti-PM/Scl antibodies in patients with SSc in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (RC), 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 with 7202 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 patients (85 from the EUSTAR registry) and compared with 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+, as compared with anti-Pm/Scl-, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of DM, 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.CONCLUSION: 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 DM, calcinosis and ILD characterized by a good functional outcome. SRC and malignancies do not seem to be part of this syndrome.",
keywords = "Adult, Autoantibodies, Europe/epidemiology, Exoribonucleases/immunology, Exosome Multienzyme Ribonuclease Complex/immunology, Female, Humans, Male, Middle Aged, Phenotype, Registries, Retrospective Studies, Scleroderma, Systemic/complications, biomarkers, scleroderma and related disorders, laboratory diagnosis, myositis and muscle disease, autoantigens and autoantibodies",
author = "Maria-Grazia Lazzaroni and Emiliano Marasco and Corrado Campochiaro and Jeska DeVries-Bouwstra and Montserrat-Ixchel Gonzalez-Perez and Jorge Rojas-Serrano and Eric Hachulla and Elisabetta Zanatta and Simone Barsotti and Federica Furini and Konstantinos Triantafyllias and Giuseppina Abignano and Marie-Elise Truchetet and {De Luca}, Giacomo and {De Langhe}, Ellen and Roger Hesselstrand and Francesca Ingegnoli and Eugenia Bertoldo and Vanessa Smith and Silvia Bellando-Randone and Hadi Poormoghim and Enrico Colombo and Angela Ceribelli and Alessio Furloni and Stefania Zingarelli and Ilaria Cavazzana and Franco Franceschini and {Del Galdo}, Francesco and Denton, {Christopher P} and Lorenzo Cavagna and Oliver Distler and Yannick Allanore and Paolo Air{\`o} and {EUSTAR Co-authors} and Susanne Ullman",
note = "{\textcopyright} The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.",
year = "2021",
month = nov,
day = "3",
doi = "10.1093/rheumatology/keab152",
language = "English",
volume = "60",
pages = "5028--5041",
journal = "Rheumatology",
issn = "1462-0324",
publisher = "Oxford University Press",
number = "11",

}

RIS

TY - JOUR

T1 - The clinical phenotype of systemic sclerosis patients with anti-PM/Scl antibodies

T2 - results from the EUSTAR cohort

AU - Lazzaroni, Maria-Grazia

AU - Marasco, Emiliano

AU - Campochiaro, Corrado

AU - DeVries-Bouwstra, Jeska

AU - Gonzalez-Perez, Montserrat-Ixchel

AU - Rojas-Serrano, Jorge

AU - Hachulla, Eric

AU - Zanatta, Elisabetta

AU - Barsotti, Simone

AU - Furini, Federica

AU - Triantafyllias, Konstantinos

AU - Abignano, Giuseppina

AU - Truchetet, Marie-Elise

AU - De Luca, Giacomo

AU - De Langhe, Ellen

AU - Hesselstrand, Roger

AU - Ingegnoli, Francesca

AU - Bertoldo, Eugenia

AU - Smith, Vanessa

AU - Bellando-Randone, Silvia

AU - Poormoghim, Hadi

AU - Colombo, Enrico

AU - Ceribelli, Angela

AU - Furloni, Alessio

AU - Zingarelli, Stefania

AU - Cavazzana, Ilaria

AU - Franceschini, Franco

AU - Del Galdo, Francesco

AU - Denton, Christopher P

AU - Cavagna, Lorenzo

AU - Distler, Oliver

AU - Allanore, Yannick

AU - Airò, Paolo

AU - EUSTAR Co-authors

A2 - Ullman, Susanne

N1 - © The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.

PY - 2021/11/3

Y1 - 2021/11/3

N2 - OBJECTIVE: To evaluate clinical associations of anti-PM/Scl antibodies in patients with SSc in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (RC), 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 with 7202 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 patients (85 from the EUSTAR registry) and compared with 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+, as compared with anti-Pm/Scl-, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of DM, 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.CONCLUSION: 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 DM, calcinosis and ILD characterized by a good functional outcome. SRC and malignancies do not seem to be part of this syndrome.

AB - OBJECTIVE: To evaluate clinical associations of anti-PM/Scl antibodies in patients with SSc in a multicentre international cohort, with particular focus on unresolved issues, including scleroderma renal crisis (RC), 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 with 7202 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 patients (85 from the EUSTAR registry) and compared with 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+, as compared with anti-Pm/Scl-, had higher frequency of muscle involvement, ILD, calcinosis and cutaneous signs of DM, 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.CONCLUSION: 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 DM, calcinosis and ILD characterized by a good functional outcome. SRC and malignancies do not seem to be part of this syndrome.

KW - Adult

KW - Autoantibodies

KW - Europe/epidemiology

KW - Exoribonucleases/immunology

KW - Exosome Multienzyme Ribonuclease Complex/immunology

KW - Female

KW - Humans

KW - Male

KW - Middle Aged

KW - Phenotype

KW - Registries

KW - Retrospective Studies

KW - Scleroderma, Systemic/complications

KW - biomarkers

KW - scleroderma and related disorders

KW - laboratory diagnosis

KW - myositis and muscle disease

KW - autoantigens and autoantibodies

UR - http://www.scopus.com/inward/record.url?scp=85111381536&partnerID=8YFLogxK

U2 - 10.1093/rheumatology/keab152

DO - 10.1093/rheumatology/keab152

M3 - Journal article

C2 - 33580257

VL - 60

SP - 5028

EP - 5041

JO - Rheumatology

JF - Rheumatology

SN - 1462-0324

IS - 11

ER -

ID: 70592385