Extracorporeal Photochemotherapy in Systemic Sclerosis and Severe Morphea

Abstract

Background: Extracorporeal photochemotherapy (EP) is a new immune-modulating therapy which combines cytapheresis and extracorporeal irradiation of leukocytes by UVA. Recently, patients with systemic sclerosis (SS) have been treated by this method, but the efficacy of EP is still controversial. Objective: Our purpose was to evaluate the efficacy and safety of EP in SS and in severe morphea. Methods: In an open study, 7 patients with SS and 2 patients with severe morphea were treated by EP and were evaluated after 6 months of treatment. No other treatments were allowed during this period. Results: Except one vasovagal reaction, EP was well tolerated in all patients. The surface of the cutaneous sclerosis was unchanged in 3 of the 7 patients with SS, whereas it was aggravated in the remaining 4 patients. There was no improvement of the visceral involvement of SS in these patients. Although the duration of SS was less than 4 years in 6 out of 7 patients, we did not observe any significant benefit from EP in SS patients. One patient with generalized morphea had stopped EP after 3 months because of loss of vascular access and could not be evaluated. In the last patient with linear morphea of the upper limbs, a reduction in the number of morphea plaques was observed, and the remaining lesions were less visible after 16 months of EP. Conclusions: The present results do not corroborate those previously published with regard to the efficiency of EP in SS of recent onset. The efficacy of ECP in morphea needs further confirmation.

Management of Severe Scleroderma with Long-Term Extracorporeal Photopheresis

“…However, abrupt discontinuation of previous immunosuppressive medication and the short treatment period of 8 months may have been at least partially responsible for these unfavourable results. In a similar study, Cribier et al [20] did not observe any significant benefit of a 6-month ECP therapy on 7 patients with systemic sclerosis of recent onset, who did not respond to conventional therapy.…”

Extracorporeal Photochemotherapy for Generalized Deep Morphea

“…10–12 A multicenter, randomized, single blind controlled trial was performed to examine the benefit from ECP in the treatment of systemic sclerosis. 8,10 After 6 months of treatment, a statistically significant reduction in skin severity scores was observed in 68% of patients receiving ECP compared with 32% of patients prescribed a regimen of D-penicillamine. 8,10 The efficacy of ECP in systemic sclerosis was further evaluated in a more recent randomized, double-blind, placebo-controlled clinical trial conducted at 16 investigational sites world-wide.…”

“…9,11 Extracorporeal photochemotherapy showed beneficial results in a single study 8 in 1 patient with linear morphea, with reduction in plaque number and decreased prominence of remaining plaques. 4 It has also been shown to be effective to varying degrees in the treatment of systemic sclerosis and chronic GVHD, diseases that share many similarities with generalized deep morphea.…”

“…2,8–12 Based on our experience using ECP in the treatment of chronic graft-vs-host disease (GVHD), and because the patient had failed all previous attempted treatments, the decision was made to attempt a 4-month trial of ECP.…”

Phototherapy of Sclerosing Skin Diseases

“…Until 10 years ago, no generally effective treatment was available for these diseases. In 1992, extracorporeal photopheresis was suggested as treatment for systemic sclerosis, but further studies are required to confirm this early report [1, 2, 3]. Two years later, psoralen + UVA, in PUVA bath photochemotherapy, was proposed as effective and safe treatment for localized scleroderma [4].…”