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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

(2017) JOURNAL OF RHEUMATOLOGY. 44(5). p.639-647
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Abstract
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.
Keywords
SYSTEMIC SCLEROSIS, SCLERODERMA, NEOPLASMS, AUTOANTIBODIES, ANTRAL VASCULAR ECTASIA, LINKED-IMMUNOSORBENT-ASSAY, RESEARCH GROUP DATABASE, OF-THE-LITERATURE, DISEASE SCLERODERMA, CLINICAL-USEFULNESS, BREAST-CANCER, ASSOCIATION, RISK, PREVALENCE

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Citation

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Chicago
Lazzaroni, Maria-Grazia, Ilaria Cavazzana, Enrico Colombo, Rucsandra Dobrota, Jasmin Hernandez, Roger Hesselstrand, Cecilia Varju, et al. 2017. “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.” Journal of Rheumatology 44 (5): 639–647.
APA
Lazzaroni, M.-G., Cavazzana, I., Colombo, E., Dobrota, R., Hernandez, J., Hesselstrand, R., Varju, C., et al. (2017). 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. JOURNAL OF RHEUMATOLOGY, 44(5), 639–647.
Vancouver
1.
Lazzaroni M-G, Cavazzana I, Colombo E, Dobrota R, Hernandez J, Hesselstrand R, et al. 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. JOURNAL OF RHEUMATOLOGY. 2017;44(5):639–47.
MLA
Lazzaroni, Maria-Grazia et al. “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.” JOURNAL OF RHEUMATOLOGY 44.5 (2017): 639–647. Print.
@article{8545931,
  abstract     = {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.},
  author       = {Lazzaroni, Maria-Grazia and Cavazzana, Ilaria and Colombo, Enrico and Dobrota, Rucsandra and Hernandez, Jasmin and Hesselstrand, Roger and Varju, Cecilia and Nagy, Gabriella and Smith, Vanessa and Caramaschi, Paola and Riccieri, Valeria and Hachulla, Eric and Balbir-Gurman, Alexandra and Chatelus, Emmanuel and Romanowska-Próchnicka, Katarzyna and Araújo, Ana Carolina and Distler, Oliver and Allanore, Yannick and Airò, Paolo},
  issn         = {0315-162X},
  journal      = {JOURNAL OF RHEUMATOLOGY},
  keywords     = {SYSTEMIC SCLEROSIS,SCLERODERMA,NEOPLASMS,AUTOANTIBODIES,ANTRAL VASCULAR ECTASIA,LINKED-IMMUNOSORBENT-ASSAY,RESEARCH GROUP DATABASE,OF-THE-LITERATURE,DISEASE SCLERODERMA,CLINICAL-USEFULNESS,BREAST-CANCER,ASSOCIATION,RISK,PREVALENCE},
  language     = {eng},
  number       = {5},
  pages        = {639--647},
  title        = {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},
  url          = {http://dx.doi.org/10.3899/jrheum.160817},
  volume       = {44},
  year         = {2017},
}

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