Extended extracorporeal photochemotherapy with extracorporeal administration of 8‐methoxypsoralen in systemic sclerosis. An Austrian single‐center study

Muellegger, Robert R.; Hofer, Angelika; Salmhofer, Wolfgang; Soyer, H. Peter; Kerl, Helmut; Wolf, Peter

doi:10.1034/j.1600-0781.2000.160505.x

Cited by 20 publications

(22 citation statements)

References 25 publications

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“…In somewhat larger numbers of patients, improvements in the skin manifestations of systemic sclerosis have been demonstrated, but no changes in, or progression of, visceral involvement during treatment was observed. 19,20 In contrast, long-term follow-up of 5 patients treated with ECP on 2 successive days per month for a mean of 59 (range 54-89) months indicated that, in selected patients with early disease, ECP produced improvements or stabilization in a range of symptoms, including skin thickening, joint mobility, pulmonary function studies, oral aperture, and functional index. 21,22 There have been 3 randomized, controlled studies of ECP in systemic sclerosis.…”

Section: Ecp May Improve Skin Involvement In Systemic Sclerosismentioning

confidence: 71%

Extracorporeal photopheresis: Past, present, and future

Knobler

Barr

Couriel

et al. 2009

Journal of the American Academy of Dermatology

113

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Section: Ecp May Improve Skin Involvement In Systemic Sclerosismentioning

confidence: 71%

Extracorporeal photopheresis: Past, present, and future

Knobler

Barr

Couriel

et al. 2009

Journal of the American Academy of Dermatology

113

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“…34 However, the influence of ECP on the progression of visceral organ involvement could not be consistently demonstrated in these uncontrolled trials. 35,36 The first randomised, observer blinded, prospective trial of ECP in SSc compared ECP with D-penicillamine, a substance with purported antifibrotic activity, in 79 patients of recent disease onset. 37 A skin score was used as the primary endpoint and 21 of 31 patients (68%) showed an improvement after 6 months of ECP compared with eight of 25 patients (32%) given D-penicillamine.…”

Section: Systemic Sclerosismentioning

confidence: 99%

Photopheresis (extracorporeal photochemotherapy)

Trautinger

Just

Knobler

2012

Photochem Photobiol Sci

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Photopheresis is a form of phototherapy where specialized equipment is used to isolate a leukocyte fraction from the peripheral blood which is then exposed to photoactivated 8-methoxypsoralen and reinfused into the patient. At the time of its invention the treatment was conceptually based on the hypothesis of T cell vaccination, i.e. the observation in experimental studies that exposure of the immune system to physically modified T cell clones leads to a specific inhibition of T cell mediated autoimmunity. Consequently, photopheresis has been tried in a variety of conditions where T cells are thought to have a critical role and has shown clinical efficacy mainly in variants of cutaneous T cell lymphomas, graft-versus-host disease, systemic sclerosis, in solid organ transplant rejection and Crohn's disease. Evidence has accumulated that alterations in antigen presentation and the generation of regulatory T cells are induced by photopheresis and might be related to the observed clinical effects. Summarizing what has been published in the 25 years since its introduction into the clinic, photopheresis to date has found its place in the treatment of the above mentioned conditions as a well tolerated treatment option that can safely be combined with other established modalities. It can be expected that further research will help refine its clinical indications and close the gaps that still exist in our knowledge on when, how, and why photopheresis works.

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“…In that trial, ECP was given on two consecutive days monthly for 12 months. In the study of Enomoto et al and the trial of Muellegger and colleagues, ECP sessions were performed every 4 weeks on two consecutive days [5,6]. They reported a minor improvement of skin changes only.…”

Section: Discussionmentioning

confidence: 95%

“…Formerly, clinical trials demonstrated the efficacy of ECP in SSc; however, they investigated clinical parameters only, such as skin or joint manifestations [3][4][5][6]. Our workgroup was the first to investigate the immunomodulatory effects of ECP in SSc patients [7].…”

Section: Introductionmentioning

confidence: 98%

The assessment of immune-regulatory effects of extracorporeal photopheresis in systemic sclerosis: a long-term follow-up study

et al. 2015

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The therapeutic options in systemic sclerosis (SSc) are limited mainly to the management of complications, and decelerating fibrosis and preventing disease progression are still great challenges. Extracorporeal photopheresis (ECP) is one of the promising therapeutic strategies in SSc; nevertheless, there is no consensus on the ideal timing and frequency of treatment cycles. In the present study, we evaluated the long-term effects of consecutive ECP treatments, and the stability of clinical and laboratory improvements. We enrolled nine patients with diffuse cutaneous SSc and performed 12 ECP cycles (24 ECP treatments) per patient in total. ECP cycles were carried out once in every 6 weeks, and each cycle consisted of two procedures. Sixteen healthy individuals served as controls for laboratory assessment. Following the sixth ECP cycle, we observed further improvement in skin score, which was confirmed by high-resolution ultrasonography as well. After the second ECP cycle, values of Tr1 and CD4+CD25(bright) Treg cells increased; however, Tr1 cells remained under control values until the 10th cycle. Suppressor activity of CD4+CD25+ Treg cells improved, while percentages of Th17 cells decreased. At the end of 12-month follow-up, we did not observe significant deterioration in skin involvement; however, improvement in laboratory parameters diminished after 12 months. If the first six ECP cycles are effective, uninterrupted continuation of treatment should be considered, which may lead to the normalization of Tr1 cell values along with further clinical improvement. Our laboratory observations indicate that immunomodulatory effect of ECP treatments lasts for 1 year only.

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Extended extracorporeal photochemotherapy with extracorporeal administration of 8‐methoxypsoralen in systemic sclerosis. An Austrian single‐center study

Abstract: Extended EXP with extracorporeal administration of 8-MOP is a safe and well tolerated treatment modality. However, it provides only (minor) improvement of skin changes of a subset of SSC patients and does not beneficially influence extracutaneous manifestations and quality of life.

Cited by 20 publications

References 25 publications

Extracorporeal photopheresis: Past, present, and future

Extracorporeal photopheresis: Past, present, and future

Photopheresis (extracorporeal photochemotherapy)

The assessment of immune-regulatory effects of extracorporeal photopheresis in systemic sclerosis: a long-term follow-up study

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