Extracorporeal photochemotherapy in progressive systemic sclerosis: a follow-up study

Area of Science:

• Immunology
• Dermatology
• Rheumatology

Background:

• Extracorporeal photochemotherapy (photopheresis) is an immune-modulating therapy with demonstrated efficacy in autoimmune disorders.
• Progressive systemic sclerosis (PSS), also known as scleroderma, is a debilitating autoimmune condition.
• The study investigates photopheresis as a treatment for PSS.

Purpose of the Study:

• To evaluate the efficacy and safety of long-term extracorporeal photochemotherapy in patients with early-onset, diffuse PSS.
• To assess the impact of photopheresis on various clinical manifestations of PSS.
• To determine the tolerability and clinical outcomes associated with extended photopheresis treatment.

Main Methods:

• Five patients with early-onset, diffuse PSS received photopheresis bi-monthly for an average of 59 months.
• Treatment involved photopheresis administered on two successive days each month.
• Patient responses were monitored over an extended period, with initial reporting at an average of 11 months.

Main Results:

• Most patients experienced improvement or stabilization in skin thickening, joint mobility, and oral aperture.
• Significant improvements were noted in functional indices and symptoms such as Raynaud's phenomenon, dyspnea, fatigue, dysphagia, arthralgias, and cutaneous ulcers.
• Renal function remained within normal limits, and 296 treatments were administered without significant toxicity.

Conclusions:

• Extended use of extracorporeal photochemotherapy is well-tolerated in patients with early-onset, diffuse PSS.
• Photopheresis may offer increasingly beneficial clinical outcomes for managing PSS.
• The study supports photopheresis as a viable long-term treatment option for PSS.