TY - JOUR T1 - Malignancies in Patients with Anti-RNA Polymerase III Antibodies and Systemic Sclerosis: Analysis of the EULAR Scleroderma Trials and Research Cohort and Possible Recommendations for Screening JF - The Journal of Rheumatology JO - J Rheumatol SP - 639 LP - 647 DO - 10.3899/jrheum.160817 VL - 44 IS - 5 AU - Maria-Grazia Lazzaroni AU - Ilaria Cavazzana AU - Enrico Colombo AU - Rucsandra Dobrota AU - Jasmin Hernandez AU - Roger Hesselstrand AU - Cecilia Varju AU - Gabriella Nagy AU - Vanessa Smith AU - Paola Caramaschi AU - Valeria Riccieri AU - Eric Hachulla AU - Alexandra Balbir-Gurman AU - Emmanuel Chatelus AU - Katarzyna Romanowska-Próchnicka AU - Ana Carolina Araújo AU - Oliver Distler AU - Yannick Allanore AU - Paolo Airò AU - EUSTAR co-authors Y1 - 2017/05/01 UR - http://www.jrheum.org/content/44/5/639.abstract N2 - Objective. To analyze the characteristics of anti-RNA polymerase III antibodies (anti-RNAP3)− positive patients with systemic sclerosis (SSc) in the European League Against Rheumatism Scleroderma Trials and Research group (EUSTAR) registry with a focus on the risk of cancer and the characteristics of malignancies, and the aim to provide guidelines about potential cancer screening in these patients.Methods. (1) Analysis of the EUSTAR database: 4986 patients with information on their anti-RNAP3 status were included. (2) Case-control study: additional retrospective data, including malignancy history, were queried in 13 participating EUSTAR centers; 158 anti-RNAP3+ cases were compared with 199 local anti-RNAP3− controls, matched for sex, cutaneous subset, disease duration, and age at SSc onset. (3) A Delphi exercise was performed by 82 experts to reach consensus for cancer screening in anti-RNAP3+ patients.Results. In the EUSTAR registry, anti-RNAP3 were associated in multivariable analysis with renal crisis and diffuse cutaneous involvement. In the case-control study, anti-RNAP3 were associated with gastric antral vascular ectasia, rapid progression of skin involvement, and malignancies concomitant to SSc onset (OR 7.38, 95% CI 1.61–33.8). When compared with other anti-RNAP3+ patients, those with concomitant malignancies had older age (p < 0.001) and more frequent diffuse cutaneous involvement (p = 0.008). The Delphi exercise highlighted the need for malignancy screening at the time of diagnosis for anti-RNAP3+ patients and tight followup in the following years.Conclusion. Anti-RNAP3+ patients with SSc have a high risk of concomitant malignancy. These results have implications for clinical practice and suggest regular screening for cancer in anti-RNAP3+ patients. ER -