FRI0330 MALIGNANCIES IN SYSTEMIC SCLEROSIS PATIENTSWITH ANTI-PM/SCL ANTIBODIES: AN EUSTAR CASE- CONTROL STUDY
Authors: Maria Grazia Lazzaroni, Stefania Zingarelli, Jeska de Vries-Bouwstra, Eric Hachulla, Elisabetta Zanatta, Simone Barsotti, Marie-Elise Truchetet, Giacomo De Luca, Vanessa Smith, Ellen De Langhe, Roger Hesselstrand, Francesca Ingegnoli, Cristian Caimmi, Silvia Bellando Randone, Enrico Colombo, Angela Ceribelli, Franco Franceschini, Yannick Allanore, Paolo Airò
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> The main clinical associations of anti-PM/Scl in Systemic Sclerosis (SSc) include calcinosis, joint and muscle involvement, interstitial lung disease (ILD), and, possibly, scleroderma renal crisis (1,2). A possible association of anti-PM/Scl with cancer was reported in single-centre SSc series (2,3), but was never analysed in large multicentre studies. Moreover, the characteristics of malignancies, in particular their temporal association with the onset of SSc, were not explored. <h3>Objectives</h3> To evaluate the association of anti-PM/Scl with malignancies in a large, international, multicentre cohort. <h3>Methods</h3> 16 EUSTAR centres provided data on anti-PM/Scl+ SSc patients in their cohorts (cases), and on anti-PM/Scl-negative controls, matched for sex, age at onset (±5 years), cutaneous subset, and disease duration (±24 months). Only SSc patients with age &gt;16 years at disease onset were included in this analysis. Anti-RNA Polymerase3+ patients were not included in controls, given the known association with synchronous cancer. Malignancies diagnosed between 2 years before and after the onset of SSc were defined as "synchronous to the onset of SSc". <h3>Results</h3> 123 anti-PM/Scl+ SSc patients and 160 matched anti-PM-Scl- controls (28% anti-Topoisomerase I, 40% anti-centromere, 32% others) were compared (Table 1): anti-Pm/Scl+ patients had a higher prevalence of myositis (p&lt;0.0001) and ILD (p&lt;0.0001), and a lower prevalence of oesophageal symptoms (p&lt;0.0001). The frequency of malignancies was not significantly different between the 2 groups (14/120 (12%) vs. 9/155 (6%), p:0.12). Only 7 malignancies synchronous to SSc onset were identified: 4 among anti-PM/Scl+ cases and 3 among controls (3 breast cancers; 3 other solid tumours; 1 multiple myeloma). Mean age at SSc onset was significantly higher in patients with synchronous malignancies compared to those without (64.9±7.2 vs. 48.4±14.5 years; p:0.003), irrespective of the anti-PM/Scl status. Conclusion: in this EUSTAR multicentre case control-study, the association of anti-Pm/Scl with malignancies in SSc patients could not be confirmed. Patients with older age at SSc onset seems to be at higher risk for synchronous malignancies, regardless the autoantibody status. <h3>References</h3> [1] Lazzaroni MG, et al. Ann Rheum Dis2018; 77:(Suppl 2),421-422. [2] Bruni C, et al. Rheumatology (Oxford). 2017;56:317-8. [3] Bernal-Bello D, et al. Autoimmun Rev2017;16:461-8. <h3>Acknowledgement</h3> Authors would like to thank GILS (Gruppo Italiano Lotta Sclerodermia) for the grant to support this project <h3>Disclosure of Interests</h3> Maria Grazia Lazzaroni: None declared, Stefania Zingarelli: None declared, Jeska de Vries-Bouwstra: None declared, Eric Hachulla Consultant for: Received consulting fees or other remuneration from Actelion, GSK, Pfizer, and Bayer, Elisabetta Zanatta: None declared, Simone Barsotti: None declared, Marie-Elise Truchetet: None declared, Giacomo De Luca Speakers bureau: Pfeizer, Sobi, Vanessa Smith: None declared, Ellen De Langhe: None declared, Roger Hesselstrand: None declared, Francesca Ingegnoli: None declared, Cristian Caimmi: None declared, Silvia Bellando Randone: None declared, Enrico Colombo: None declared, Angela Ceribelli: None declared, Franco Franceschini: None declared, Yannick Allanore Grant/research support from: Inventiva, F Hoffman La-Roche, Sanofi, BMS, Pfizer, Consultant for: Actelion, Bayer, BMS, Boehringer, Roche, Sanofi, Paolo Airò: None declared