The Influence of Extracorporeal Photopheresis on Skin Sclerosis

NCT04752397 | Completed

• 1 other identifier: CRC-A-34
• Study Type: observational
• Target: 6
• Locations: 1 country
• Sites: 1

Brief Summary

Extracorporeal photopheresis (ECP), also known as extracorporeal photoimmunotherapy or photochemotherapy, is a leukapheresis-based therapy that has been in clinical use for over three decades after receiving FDA approval in 1988. Extracorporeal photopheresis was initially used for the treatment of T-cell lymphoma. Since its introduction, indications for initiating ECP were continuously extended to the treatment of Graft-versus-Host Disease (GvHD), systemic sclerosis, and in the field of solid organ transplantation. There is also evidence supporting the use of ECP in generalized morphea, a form of scleroderma limited to the skin, and in eosinophilic fasciitis, which is a rare, localized fibrosing disorder of the fascia. Concluding the results of the published studies, there is evidence that ECP has a positive effect on fibrosing disorders of the skin. Furthermore, in clinical practice, it has been observed that patients with systemic sclerosis, who undergo ECP treatment, show improvement of the skin lesions or a deceleration in the formation progress of such lesions during the therapy. Same findings can be observed in patients with sclerotic skin lesions of the skin, for example in the context of a GvHD. There are no clinical studies so far that describe these processes using objective measuring methods. Furthermore, the mechanism of action of ECP in systemic sclerosis and other fibrosing disorders with skin manifestations, has not yet been conclusively clarified. Serological markers for monitoring the progress of the therapy and determining the prognosis are also missing. Thus, a consensus regarding the frequency and duration of ECP for the therapy of systemic scleroderma or sclerotic diseases has not yet been reached. This study aims at evaluating the influence of Extracorporeal Photopheresis on the quality and functionality of sclerotic skin lesions assessed by several objective methods. Furthermore, potential biomarkers, which are being investigated in current studies, are to be determined in order to evaluate the influence of ECP on those biomarkers and better understand the mechanism of action of ECP on systemic sclerosis and fibrosing disorders involving the skin.

Conditions

Scleroderma, Systemic; Graft Vs Host Disease; Eosinophilic Fasciitis; Morphea; Skin Sclerosis

Keywords

Extracorporeal photopheresis; Systemic Sclerosis; Morphea; Graft Vs Host Disease; Eosinophilic Fasciitis; Transepidermal water loss; Stratum corneum hydration; Skin firmness; High frequency ultrasonography; Skin surface sebum; Skin thickness; Biomarkers

Outcome Measures

Primary Outcomes (6)

• Modified Rodnan Skin score

  The skin thickening is being assessed by palpation of the skin in 17 areas of the body using a 0-3 scale, where 0 = normal, 1 = mild thickness, 2 = moderate thickness and 3 = severe thickness (total score=51).

  Week 4 ± 2

• Modified Rodnan Skin score

  The skin thickening is being assessed by palpation of the skin in 17 areas of the body using a 0-3 scale, where 0 = normal, 1 = mild thickness, 2 = moderate thickness and 3 = severe thickness (total score=51).

  Week 8 ± 2

• Modified Rodnan Skin score

  The skin thickening is being assessed by palpation of the skin in 17 areas of the body using a 0-3 scale, where 0 = normal, 1 = mild thickness, 2 = moderate thickness and 3 = severe thickness (total score=51).

  Week 12 ± 2

• Modified Rodnan Skin score

  The skin thickening is being assessed by palpation of the skin in 17 areas of the body using a 0-3 scale, where 0 = normal, 1 = mild thickness, 2 = moderate thickness and 3 = severe thickness (total score=51),

  Week 16 ± 2

• Modified Rodnan Skin score

  The skin thickening is being assessed by palpation of the skin in 17 areas of the body using a 0-3 scale, where 0 = normal, 1 = mild thickness, 2 = moderate thickness and 3 = severe thickness (total score=51).

  Week 20 ± 2

• Modified Rodnan Skin score

  The skin thickening is being assessed by palpation of the skin in 17 areas of the body using a 0-3 scale, where 0 = normal, 1 = mild thickness, 2 = moderate thickness and 3 = severe thickness (total score=51).

  Week 24 ± 2

Secondary Outcomes (47)

• Skin thickness at control skin area

  Week 12 ± 2

• Skin thickness at lesional skin area

  Week 12 ± 2

• Skin thickness at control skin area

  Week 24 ± 2

• Skin thickness at lesional skin area

  Week 24 ± 2

• Transepidermal water loss (TEWL) at control skin area

  Week 12 ± 2

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients who receive ECP in the Clinic of Dermatology, Venerology and Allergology - Charité - Universitätsmedizin Berlin.

You may qualify if:

• Systemic sclerosis, morphea, sclerodermiform GvHD or eosinophilic fasciitis, with duration less than 5 years.
• Ability and willingness to both understand and carry out the study requirements

You may not qualify if:

• \. Participation in another study, currently or in the previous four weeks.

Sponsors & Collaborators

• Charite University, Berlin, Germany: lead

Study Sites (1)

Charité-Universitätsmedizin Berlin

Berlin, 10117, Germany

Location

Related Publications (11)

• Cho A, Jantschitsch C, Knobler R. Extracorporeal Photopheresis-An Overview. Front Med (Lausanne). 2018 Aug 27;5:236. doi: 10.3389/fmed.2018.00236. eCollection 2018.

  PMID: 30211164 BACKGROUND

• Zhou XA, Choi J. Photopheresis: Advances and Use in Systemic Sclerosis. Curr Rheumatol Rep. 2017 Jun;19(6):31. doi: 10.1007/s11926-017-0662-8.

  PMID: 28466383 BACKGROUND

• Knobler R, Berlin G, Calzavara-Pinton P, Greinix H, Jaksch P, Laroche L, Ludvigsson J, Quaglino P, Reinisch W, Scarisbrick J, Schwarz T, Wolf P, Arenberger P, Assaf C, Bagot M, Barr M, Bohbot A, Bruckner-Tuderman L, Dreno B, Enk A, French L, Gniadecki R, Gollnick H, Hertl M, Jantschitsch C, Jung A, Just U, Klemke CD, Lippert U, Luger T, Papadavid E, Pehamberger H, Ranki A, Stadler R, Sterry W, Wolf IH, Worm M, Zic J, Zouboulis CC, Hillen U. Guidelines on the use of extracorporeal photopheresis. J Eur Acad Dermatol Venereol. 2014 Jan;28 Suppl 1(Suppl 1):1-37. doi: 10.1111/jdv.12311.

  PMID: 24354653 BACKGROUND

• Hassani J, Feldman SR. Phototherapy in Scleroderma. Dermatol Ther (Heidelb). 2016 Dec;6(4):519-553. doi: 10.1007/s13555-016-0136-3. Epub 2016 Aug 12.

  PMID: 27519050 BACKGROUND

• Gabrielli A, Avvedimento EV, Krieg T. Scleroderma. N Engl J Med. 2009 May 7;360(19):1989-2003. doi: 10.1056/NEJMra0806188. No abstract available.

  PMID: 19420368 BACKGROUND

• Guggino G, Lo Pizzo M, Di Liberto D, Rizzo A, Cipriani P, Ruscitti P, Candore G, Gambino CM, Sireci G, Dieli F, Giacomelli R, Triolo G, Ciccia F. Interleukin-9 over-expression and T helper 9 polarization in systemic sclerosis patients. Clin Exp Immunol. 2017 Nov;190(2):208-216. doi: 10.1111/cei.13009. Epub 2017 Aug 23.

  PMID: 28681919 BACKGROUND

• Li L, Zhu H, Zuo X. Interleukin-33 in Systemic Sclerosis: Expression and Pathogenesis. Front Immunol. 2018 Nov 15;9:2663. doi: 10.3389/fimmu.2018.02663. eCollection 2018.

  PMID: 30498500 BACKGROUND

• Sacchetti C, Bai Y, Stanford SM, Di Benedetto P, Cipriani P, Santelli E, Piera-Velazquez S, Chernitskiy V, Kiosses WB, Ceponis A, Kaestner KH, Boin F, Jimenez SA, Giacomelli R, Zhang ZY, Bottini N. PTP4A1 promotes TGFbeta signaling and fibrosis in systemic sclerosis. Nat Commun. 2017 Oct 20;8(1):1060. doi: 10.1038/s41467-017-01168-1.

  PMID: 29057934 BACKGROUND

• Matsushita T, Takehara K. An update on biomarker discovery and use in systemic sclerosis. Expert Rev Mol Diagn. 2017 Sep;17(9):823-833. doi: 10.1080/14737159.2017.1356722. Epub 2017 Jul 25.

  PMID: 28730919 BACKGROUND

• Romano C, Rubegni P, De Aloe G, Stanghellini E, D'Ascenzo G, Andreassi L, Fimiani M. Extracorporeal photochemotherapy in the treatment of eosinophilic fasciitis. J Eur Acad Dermatol Venereol. 2003 Jan;17(1):10-3. doi: 10.1046/j.1468-3083.2003.00587.x.

  PMID: 12602960 BACKGROUND

• Abraham DJ, Krieg T, Distler J, Distler O. Overview of pathogenesis of systemic sclerosis. Rheumatology (Oxford). 2009 Jun;48 Suppl 3:iii3-7. doi: 10.1093/rheumatology/ken481.

  PMID: 19487220 BACKGROUND

Biospecimen

Retention: NONE RETAINED

Blood sample

MeSH Terms

Conditions

Scleroderma, Systemic; Graft vs Host Disease; Eosinophilic Fasciitis; Scleroderma, Localized

Condition Hierarchy (Ancestors)

Connective Tissue Diseases; Skin and Connective Tissue Diseases; Skin Diseases; Immune System Diseases

Study Officials

• Ulrike Blume-Peytavi, Prof. MD

  ulrike.blume-peytavi@charite.de

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Prof. Dr. Ulrike Blume-Peytavi

Study Record Dates

• First Submitted: February 9, 2021
• First Posted: February 12, 2021
• Study Start: February 12, 2021
• Primary Completion: August 17, 2021
• Study Completion: September 6, 2021
• Last Updated: November 17, 2022

Record last verified: 2022-11

Locations

DE (1)