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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

Lazzaroni, Maria Grazia; Cavazzana, Ilaria; Colombo, Enrico; Dobrota, Rucsandra; Hernandez, Jasmin; Hesselstrand, Roger LU ; Varju, Cecilia; Nagy, Gabriella; Smith, Vanessa and Caramaschi, Paola, et al. (2017) In Journal of Rheumatology 44(5). p.639-647
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,... (More)

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.

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published
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keywords
Autoantibodies, Neoplasms, Scleroderma, Systemic sclerosis
in
Journal of Rheumatology
volume
44
issue
5
pages
9 pages
publisher
J Rheumatol Publ Co
external identifiers
  • scopus:85018318817
  • wos:000401094300017
ISSN
0315-162X
DOI
10.3899/jrheum.160817
language
English
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yes
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4f64e2bf-818a-4541-bf9a-5b4138a53b00
date added to LUP
2017-05-24 16:01:21
date last changed
2018-04-29 04:39:07
@article{4f64e2bf-818a-4541-bf9a-5b4138a53b00,
  abstract     = {<p>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 &lt; 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.</p>},
  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 Carolina Araújo, Ana and Distler, Oliver and Allanore, Yannick and Airo, Paolo},
  issn         = {0315-162X},
  keyword      = {Autoantibodies,Neoplasms,Scleroderma,Systemic sclerosis},
  language     = {eng},
  month        = {05},
  number       = {5},
  pages        = {639--647},
  publisher    = {J Rheumatol Publ Co},
  series       = {Journal of Rheumatology},
  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},
}