Table 4.

Results of the Delphi exercise. Degree of agreement with the proposed recommendations about screening for synchronous cancer in anti-RNAP3+ patients with SSc among 82 EUSTAR experts (1–10, with 10 = fully agree).

Proposed RecommendationsMeanSD
Screening for synchronous malignancies is recommended8.731.70
Screening for synchronous breast cancer in female patients is recommended with mammography (or US/MRI when needed)9.021.35
Screening for other malignancies should be guided by clinical suspicion and patient age. Noninvasive tests (e.g., fecal occult blood, gynecological evaluation, prostatic-specific antigen) may be considered in all patients8.631.48
Serum tumor markers are NOT useful for screening in every patient8.592.18
A period of 2–5 yrs of tight surveillance for cancer is recommended8.262.01
If the screening tests for cancer performed at the diagnosis of scleroderma are negative, tests for breast cancers should be repeated (e.g., annually); other tests should be repeated in case of clinical suspicion7.902.14
PET/CT may be considered when unspecific systemic signs suggest the possible presence of neoplasms8.002.02
Further tight surveillance for cancer is not recommended after a period of 2–5 yrs7.102.27
Prospective studies are needed to clarify how long the tight surveillance should last and which examinations are most indicated for screening of cancer in these patients9.561.04
  • SSc: systemic sclerosis; EUSTAR: European League Against Rheumatism Scleroderma Trials and Research group; US: ultrasound; MRI: magnetic resonance imaging; PET: positron emission tomography; CT: computed tomography; anti-RNAP3: anti-RNA polymerase III antibodies.