Photopheresis: Advances and Use in Systemic Sclerosis

  • Xiaolong Alan Zhou
  • Jaehyuk Choi
Scleroderma (J Varga, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Scleroderma


Purpose of Review

Extracorporeal photochemotherapy (photopheresis, ECP) is a cell-based immunomodulatory treatment that separates leukocytes from peripheral blood, exposes them to a photosensitizing agent followed by ultraviolet A light, and then reinfuses them back into the patient. ECP has been found to be effective for graft versus host disease, transplant rejection, and various autoimmune diseases. The mechanism is not well understood but studies have shown clinical benefit in the treatment of systemic sclerosis (SSc). This review examines the ECP technique, advances in our knowledge of its mechanism, and the data supporting its use in SSc-like fibrosing diseases and in SSc itself.

Recent Findings

Multiple lines of evidence support ECP use in SSc. ECP generates apoptotic cells and dendritic cells, induces production of anti-inflammatory cytokines, and increases regulatory T cell numbers. Clinical studies have generally demonstrated improvement, especially the skin, in SSc patients receiving ECP.


ECP may be an effective and safe procedure for the treatment of SSc.


Systemic sclerosis SSc Scleroderma Extracorporeal photopheresis ECP Extracorporeal photochemotherapy 


Compliance with Ethics Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  1. 1.Department of DermatologyNorthwestern University Feinberg School of MedicineChicagoUSA
  2. 2.Department of Biochemistry and Molecular GeneticsNorthwestern University Feinberg School of MedicineChicagoUSA
  3. 3.Robert H. Lurie Comprehensive Cancer CenterNorthwestern UniversityChicagoUSA

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