EUSTAR Registry - Cancer in Systemic Sclerosis Save

Cancer is a major cause of mortality in systemic sclerosis (SSc).  A nested case-control study within EUSTAR registry shows distinct patterns of cancer related temporally to SSc, serologic profiles and traditional cancer risk factors.

Cancers were classified as synchronous to SSc onset (±3 years), subsequent (>3 years after), or previous (>3 years before) and were correlated with clinical, treatments, and serologies (specifically anti-RNA polymerase III (POLR3)-positivity). 

A total of 454 SSc patients with cancer (29% synchronous, 51% subsequent, 20% previous),  were compared to 454 matched controls were identified. Mean age was 55 years, disease duration 5 years; 88% were female, 27.5% diffuse SSc; 30.5% had interstitial lung disease (ILD), and 32% were anti-topoisomerase, and 10% were anti-POLR3 positive. 

Synchronous cancers were significantly associated with: 

• anti-POLR3 (OR 2.06)
• U1RNP (OR 3.56)
• Smoking (OR 1.57)
• but negatively with digital ulcers (OR 0.55, 0.31-0.93). 

Subsequent cancers were significantly associated with: 

• Inversely with Calcinosis (OR 0.42, 0.17-0.93).
• Breast cancer showed was anti-POLR3 and anti-PM/Scl
• Lung cancer was mainly associated with ILD (OR 2.00), anti-topoisomerase (OR 2.61), and smoking.

Malignancy worsened overall survival, particularly when subsequent. Radiation therapy did not impact mortality or new-onset ILD.

Screening for cancer, at SSc diagnosis and subsequently is advised as cancers up are major determinants of mortality.