Maria-Grazia Lazzaroni1,2, Emiliano Marasco3, Corrado Campochiaro4,5, Jeska DeVries-Bouwstra6, Montserrat-Ixchel Gonzalez-Perez7, Jorge Rojas-Serrano7, Eric Hachulla8, Elisabetta Zanatta9, Simone Barsotti10, Federica Furini11, Konstantinos Triantafyllias12, Giuseppina Abignano13, Marie-Elise Truchetet14, Giacomo De Luca4, Ellen De Langhe15, Roger Hesselstrand16, Francesca Ingegnoli17, Eugenia Bertoldo18, Vanessa Smith19, Silvia Bellando-Randone20, Hadi Poormoghim21, Enrico Colombo22, Angela Ceribelli23, Alessio Furloni2, Stefania Zingarelli1, Ilaria Cavazzana1, Franco Franceschini1,2, Francesco Del Galdo13, Christopher P Denton5, Lorenzo Cavagna3, Oliver Distler24, Yannick Allanore25, Paolo Airò1. 1. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili of Brescia. 2. Department of Clinical and Experimental Sciences, University of Brescia, Brescia. 3. Division of Rheumatology, Hospital Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo Foundation of Pavia, University of Pavia, Pavia. 4. Unit of Immunology, Rheumatology, Allergy and Rare Diseases, Istituto di Ricovero e Cura a Carattere Scientifico San Raffaele Scientific Institute and Vita-Salute San Raffaele University, Milan, Italy. 5. Royal Free Hospital and University College London Medical School, London, UK. 6. Department of Rheumatology, Leiden University Medical Centre, Leiden, the Netherlands. 7. Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, México. 8. Department of Internal Medicine, University Lille Nord-de-France, Lille, France. 9. Rheumatology Unit, Department of Medicine, University of Padova, Padova. 10. Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa. 11. Unità Operativa Complessa Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. 12. ACURA Centre for Rheumatic Diseases, Bad Kreuznach, Germany. 13. Leeds Institute of Rheumatic and Musculoskeletal Medicine and National Institute for Health Research Biomedical Research Centre, Leeds, UK. 14. Department of Rheumatology and ImmunoConcept, University Hospital, Bordeaux, France. 15. Department of Rheumatology, University Hospitals Leuven, Leuven, Belgium. 16. Department of Clinical Sciences, Section of Rheumatology, Lund University, Lund, Sweden. 17. Division of Clinical Rheumatology, Department of Clinical Sciences and Community Health, Research Centre for Adult and Paediatric Rheumatic Diseases, ASST Pini-Centro Traumatologico Ortopedico, Università Degli Studi di Milano, Milano. 18. Rheumatology Unit, Azienda Ospedaliero Universitaria Integrata, Verona, Italy. 19. Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium. 20. Department of Biomedicine, Division of Rheumatology AOUC and Department of Clinical and Experimental Medicine, University of Florence, Florence, Italy. 21. Scleroderma Study Group, Department of Rheumatology, Firoozgar Hospital, Tehran, Iran. 22. University of Sassari, Sassari. 23. Humanitas Clinical and Research Centre, Istituto di Ricovero e Cura a Carattere Scientifico, Rozzano, Italy. 24. Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland. 25. Department of Rheumatology, University of Paris, Cochin Hospital and Institut national de la santé et de la recherche médicale U1016, Paris, France.
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.
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.