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FRI0293 Screening for Malignancies in Systemic Sclerosis Patients Positive for anti-RNA Polymerase III Antibodies: A Delphi Exercise among EUSTAR Experts
  1. M.G. Lazzaroni1,
  2. I. Cavazzana2,
  3. P. Airo'2,
  4. on behalf of EUSTAR co-authors
  1. 1Spedali Civili and University of Brescia
  2. 2Spedali Civili of Brescia, Brescia, Italy


Background A temporal relationship between the onset of systemic sclerosis (SSc) and malignancies has been described in patients with anti-RNA polymerase III antibodies (anti-RNAP). Our analysis of the EUSTAR cohort (1) provided further information, highlighting the relationship of anti-RNAP with malignancies synchronous (±2 years) to the onset of SSc (OR 3.85, 95%CI 1.3–10.9). In particular, an increased rate of synchronous breast cancer was found (OR 20.2, 95%CI 1.4–355). These results suggest the possible need of a cancer screening program for these patients.

Objectives To draw up a possible list of recommendations to guide the clinicians in every-day practice to screen anti-RNAP+ SSc patients for cancer.

Methods A web-based Delphi approach was used to address the questions concerning possible screening for malignancies in anti-RNAP+ SSc patients. Prior to the first stage of Delphi exercise, the number of anti- RNAP+ patients needed to screen to find one synchronous malignancy was calculated using the results of the EUSTAR cohort. All EUSTAR centres were contacted: information on the analysis of the EUSTAR cohort was presented and centres were asked to participate to the exercise, voting on the opportunity of a screening for malignancies in anti-RNAP+ patients and its modality. In the second stage, questions that remained unsolved at the first step, and new suggestions from experts were re-proposed. Finally, possible recommendations were draft, and participants were asked to vote on them in stage 3 of the Delphi exercise. The level of agreement with each statement was voted using a 10-point visual analogue scale (10: fully agree).

Results The number needed to screen to detect one synchronous malignancy in anti-RNAP+ patients in the EUSTAR cohort was 15; the number needed to screen to detect synchronous breast cancer was 22 (or 17, considering only female patients). Eighty-two experts from EUSTAR centers participated to the third stage of the Delphi exercise voting on possible recommendations on screening for synchronous cancer in anti-RNAP+ SSc patients: the results are shown in Table 1.

Conclusions Most EUSTAR experts who participated to the Delphi exercise would recommend screening for synchronous malignancies at the moment of diagnosis in anti-RNAP+ SSc patients. In particular, screening for synchronous breast cancer in female patients, and for other malignancies, guided by clinical suspicion and patient age, would be recommended. Non-invasive tests may be considered in all patients. Although most experts agreed on the proposed statements, some degree of discordance was found on the use of PET/CT and the program of follow-up in subsequent years: in particular on how long should the tight surveillance lasts, and which exams are most indicated to be repeated. The experts agreed that prospective studies are needed to clarify these issues.

  1. M.G. Lazzaroni et al. Anti-RNA Polymerase III Antibodies in Patients with Systemic Sclerosis: A Eustar Multicenter Collaborative Study. Ann Rheum Dis 2015;74:Suppl 2 88 doi:10.1136/annrheumdis-2015-eular.1728

Disclosure of Interest None declared

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