Extracorporeal Photochemotherapy in Progressive Systemic Sclerosis

Spaltro, F X Di; Cottrill, Carolyn M.; Cahill, Carol; Degnan, Edward J.; Mulford, G J; Scarborough, Dwight A.; Franks, Andrea; Klainer, Albert S.; Bisaccia, Emil

doi:10.1111/j.1365-4362.1993.tb02811.x

Cited by 41 publications

(33 citation statements)

References 12 publications

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“…EP is probably not indicated in severe SS with respiratory or renal failure. Except in the study by Di Spaltro et al [11 ], the possible efficacy of EP in improving the quality of life of the patients is unknown. If EP can be considered to be effective in reducing the cutaneous sclerosis of certain patients with SS, further studies are still necessary to determine (1) the exact profile of EP responders and the indications of the treatment, (2) for how long the treatment should be contin ued and (3) the long-term efficay of EP.…”

Section: Resultsmentioning

confidence: 96%

“…As did Zachariae et al [6,7], Di Spaltro et al [11], we studied only a few patients, without a control group, but the same EP procedure [12j was used in all four studies. We were very surprised to observe a worsening of the disease in patients who shared all the postulated criteria for being responders to EP (patients 3 and 5 especially).…”

Section: Number O F Patientsmentioning

confidence: 99%

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Extracorporeal Photochemotherapy in Systemic Sclerosis and Severe Morphea

et al. 1995

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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.

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Section: Resultsmentioning

confidence: 96%

Section: Number O F Patientsmentioning

confidence: 99%

Extracorporeal Photochemotherapy in Systemic Sclerosis and Severe Morphea

et al. 1995

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“…However, this study has been criticized due to the short wash-out phase of 1 week prior to ECP, the lack of long-term follow-up on photopheresis and the absence of a significant difference between ECP and D -penicillamine at the 10-month evaluation point. In another study, DiSpaltro et al [10] reported overall improvement or stabilization in 8 of 9 patients treated with ECP for a rather short time of 7 months. In contrast, Zachariae et al [19] found no significant improvement after treating 8 systemic scleroderma patients with ECP.…”

Section: Discussionmentioning

confidence: 99%

“…Preliminary studies were also conducted in patients with other skin disorders [6, 7, 8, 9]. ECP was shown to act beneficially in scleroderma patients [10, 11, 12] and was found to be superior to D -penicillamine in a controlled study [11]. …”

Section: Introductionmentioning

confidence: 99%

Management of Severe Scleroderma with Long-Term Extracorporeal Photopheresis

et al. 1998

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Background: The management of systemic sclerosis remains unsatisfactory. Thus far, the action of extracorporeal photopheresis (ECP) in severe systemic scleroderma has been evaluated in short-term studies, and only limited experience has been obtained with long-term application. Objective: The aim of the present study was to evaluate prospectively the long-term effect of ECP in a group of 16 patients suffering from severe scleroderma, showing visceral involvement and progressive clinical course. Methods: Fourteen patients with systemic scleroderma involving several organs, 1 with CREST syndrome and another with scleroderma-myositis overlap syndrome were treated with ECP over a period of 6–45 months. In 3 cases, γ-IFN was additionally administered. Skin and visceral involvement were assessed by evaluating a series of clinical criteria and results from laboratory, imaging and functional tests. Results: Overall, clear improvement was encountered in 6 patients, mixed response in 2, stable disease in 3 and continuing progressive course in 5 patients. Four out of 6 patients with improvement were treated with ECP early after onset of scleroderma (≤2 years), whereas all patients with a progressive course under ECP had had scleroderma for longer than 2 years. Immunosuppressive drugs previously administered could be reduced or fully withdrawn under ECP treatment in 5 patients, but additional oral medication was introduced in 4 patients due to disease progression. Addition of γ-IFN to ECP did not reveal further benefit. No side-effects were recorded under ECP treatment. Conclusions: Based on this observation, we believe that long-term ECP represents an effective treatment modality in severe scleroderma particularly when started early, with stabilization of the disease course and partial remission of the cutaneous findings, whereas visceral involvement, if present, may rarely improve.

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“…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. The first was a large, single-blind (assessor blinded) comparison of ECP and D-penicillamine in 79 patients with systemic sclerosis of recent onset.…”

Section: Ecp May Improve Skin Involvement In Systemic Sclerosismentioning

confidence: 99%