Treatment of patients with systemic sclerosis with extracorporeal photochemotherapy (photopheresis)

Enomoto, Dory N.H.; Mekkes, Jan R.; Bossuyt, Patrick M.; Yongd, Si-La; Outd, Theo A.; Hoekzema, R.; Rie, Menno A. de; Schellekens, P. T. A.; Berge, Ineke J. M. ten; Borgieb, Corianne A.J.M. de; Bos, Jan D.

doi:10.1016/s0190-9622(99)70246-x

Cited by 79 publications

(71 citation statements)

References 50 publications

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“…Although various treatments (glucocorticoids, chlorambucil, MTX, azathioprine, 5-FU, ATG, mycophenolate mofetil, interferon-a, interferon-g, cyclosporine A, D-penicillamine and extracorporeal photopheresis) have been used to treat SSc, [19][20][21][22][23][24][25][26][27][28][29][30] currently there is no known therapy that can change the natural history of SSc. Immune suppression with CY at 1-2.5 mg/kg/day orally (with low dose corticosteroids) or 750-1000 mg/m 2 intravenously monthly for 6-12 months has been reported to be effective in treatment of scleroderma alveolitis, [31][32][33][34][35][36][37][38] but the benefit to other features and survival advantage are not clear.…”

Section: Discussionmentioning

confidence: 99%

Autologous non-myeloablative hematopoietic stem cell transplantation in patients with systemic sclerosis

Oyama

Barr

Statkute

et al. 2007

Bone Marrow Transplant

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Autologous hematopoietic stem cell transplantation (HSCT) utilizing a myeloablative regimen containing total body irradiation has been performed in patients with systemic sclerosis (SSc), but with substantial toxicity. We, therefore, conducted a phase I non-myeloablative autologous HSCT study in 10 patients with SSc and poor prognostic features. PBSC were mobilized with CY and G-CSF. The PBSC graft was cryopreserved without manipulation and re-infused after the patient was treated with a non-myeloablative conditioning regimen of 200 mg/kg CY and 7.5 mg/kg rabbit antithymocyte globulin. There was a statistically significant improvement of modified Rodnan skin score whereas cardiac (ejection fraction, pulmonary arterial pressure), pulmonary function (DLCO) and renal function (creatinine) remained stable without significant change. One patient with advanced disease died 2 years after the transplant from progressive disease. After median follow-up of 25.5 months, the overall and progression-free survival rates are 90 and 70% respectively. Autologous HSCT utilizing a non-myeloablative conditioning regimen appears to result in improved skin flexibility similar to a myeloablative TBI containing regimen, but without the toxicity and risks associated with TBI.

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Section: Discussionmentioning

confidence: 99%

Autologous non-myeloablative hematopoietic stem cell transplantation in patients with systemic sclerosis

Oyama

Barr

Statkute

et al. 2007

Bone Marrow Transplant

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“…Confirmatory evidence for a benefit of ECP in a randomized clinical trial setting is therefore still lacking. By comparison, scleroderma is a category III indication for TPE (see Scleroderma/Progressive Systemic Sclerosis fact sheet) [4,12,[30][31][32].…”

Section: Scleroderma/progressive Systemic Sclerosismentioning

confidence: 99%

Category IV indications for therapeutic apheresis—ASFA fourth special issue

Shaz

Linenberger

Bandarenko

et al. 2007

J of Clinical Apheresis

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The American Society for Apheresis (ASFA) Committee on Clinical Applications systematically and critically reviews published information on the use of therapeutic apheresis in clinical practice. On the basis of this review, selected diseases are assigned one of five categories (category I, II, III, IV, and P). The diseases, which were classified as category IV indications, and the rationale for such assignment are reviewed in this article. The diseases assigned to category I, II, III, and newly created category P are discussed in a separate article in this issue.

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“…It represents the induration or loss of elasticity assessed in a body area. The local skin score is estimated by pinching a skin fold in one area and scoring it on a scale of 0 to 3, indicating, respectively, no sclerosis to severe sclerosis (Enomoto et al, 1999). The mean skin score is the average of the total body surface subdivided into 74 areas.…”

mentioning

confidence: 99%

Dermal Organization in Scleroderma: The Fast Fourier Transform and the Laser Scatter Method Objectify Fibrosis in Nonlesional as well as Lesional Skin

Vries

Enomoto

Marle

et al. 2000

Laboratory Investigation

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SUMMARY:Scleroderma, a chronic, progressive disorder, is characterized by dermal fibrosis with collagen bundles orientated parallel to the epidermis. Simple objective parameters to evaluate disease progression and therapies are needed. We describe two methods, the laser scatter method and the fast Fourier transform (FFT), to measure collagen bundle orientation and spacing. Lesional sclerodermic skin (LS), nonlesional sclerodermic skin (nonLS), and control skin (CS) sections were evaluated for orientation ratio using the laser scatter method. The FFT was used to calculate orientation ratio, variation, and spacing of collagen bundles. Parameters were correlated with local and mean skin score measurements, on a scale of 0 (normal) to 3 (severely sclerotic). With both the laser scatter method and the FFT, orientation ratios of LS (respectively, 2.16 Ϯ 0.33 and 1.83 Ϯ 0.62) were significantly higher than CS (respectively, 1.70 Ϯ 0.35 and 1.38 Ϯ 0.15). NonLS orientation ratios (respectively, 1.92 Ϯ 0.15 and 1.48 Ϯ 0.44) were between LS and CS ratios. Orientation variation and bundle spacing of LS (respectively, 57.3 Ϯ 19.4 and 15.7 Ϯ 5.6 m) were significantly reduced compared to CS (respectively, 73.8 Ϯ 15.0 and 18.9 Ϯ 1.9 m). NonLS orientation ratios (respectively, 57.2 Ϯ 29.0 and 15.6 Ϯ 6.1 m) were similar to LS. Bundles in LS are more parallel, show less variation in orientation, and are more densely packed than in CS. There was a linear correlation between mean skin score and orientation ratio. Local skin score was not linearly correlated to orientation ratio. Our findings suggest that nonLS dermis without clinical sclerosis already shows fibrotic characteristics. Both techniques were easy to use and suitable for objectifying dermal fibrosis in scleroderma lesions. FFT is more accurate and reproducible than the laser scatter method and allows simultaneous pathological evaluation of the location of the analyzed tissue sections. Future studies will need to focus on the correlation between clinical disease severity and collagen bundle characteristics. (Lab Invest 2000, 80:1281-1289.

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Treatment of patients with systemic sclerosis with extracorporeal photochemotherapy (photopheresis)

Cited by 79 publications

References 50 publications

Autologous non-myeloablative hematopoietic stem cell transplantation in patients with systemic sclerosis

Autologous non-myeloablative hematopoietic stem cell transplantation in patients with systemic sclerosis

Category IV indications for therapeutic apheresis—ASFA fourth special issue

Dermal Organization in Scleroderma: The Fast Fourier Transform and the Laser Scatter Method Objectify Fibrosis in Nonlesional as well as Lesional Skin

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