Incidence of lung cancer in patients with systemic sclerosis treated with extracorporeal photopheresis

Photodermatol Photoimmunol Photomed. 2015 Jul;31(4):175-83. doi: 10.1111/phpp.12155. Epub 2015 Jan 15.


Background: Extracorporeal photopheresis (ECP) improves skin sclerosis in systemic sclerosis (SSc) patients. SSc is associated with an increased risk of lung cancer. As ECP is supposed to exert immunomodulatory effects, a possible impact of ECP on the incidence of lung cancer in SSc patients was evaluated.

Methods: Seventy-one SSc patients treated with ECP at the Photopheresis Unit of the Department of Dermatology at the Medical University of Vienna between 1991 and 2013 were analyzed retrospectively.

Results: We calculated a standardized incidence ratio (SIR) for lung cancer in ECP-treated SSc patients of 2.34 [95% confidence interval (CI) 1.63-2.49]. This is in accordance with recent meta-analyses demonstrating a significantly enhanced risk of lung carcinoma in SSc patients. Comparison of the lung cancer risks of these patients with our ECP-treated patients revealed that ECP has no influence. Each patient with lung carcinoma had previously been diagnosed with lung involvement of the non-specific interstitial pneumonitis (NSIP) type.

Conclusion: We confirm that SSc patients are at significantly increased risk for lung cancer. However, ECP does not influence this risk. NSIP may be a risk factor for lung cancer in SSc patients.

Keywords: extracorporeal photopheresis; lung cancer; systemic sclerosis.

MeSH terms

  • Female
  • Humans
  • Incidence
  • Lung Neoplasms / epidemiology*
  • Lung Neoplasms / pathology
  • Male
  • Middle Aged
  • Photopheresis / adverse effects*
  • Risk Factors
  • Scleroderma, Systemic / epidemiology*
  • Scleroderma, Systemic / pathology
  • Scleroderma, Systemic / therapy*