Management of Severe Scleroderma with Long-Term Extracorporeal Photopheresis

Abstract

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 Immunosuppressive drugs previously administered could be reduced or fully withdrawn under ECP treatment in 5 patients, but additional oral medication was introduced in 4 patients due to disease progression.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.

Extrakorporale Photopherese

“…Auch konnte nachgewiesen werden, dass durch Photopherese nicht nur eine Verbesserung der kutanen Symptomatik der Sklerodermie erreicht wurde, sondern es kam auch zu einer signifikanten Verbesserung der Gelenksmitbeteiligung im Rahmen der Grundkrankheit [3,14].…”

Long-Term Extracorporeal Photoimmunotherapy for Treatment of Chronic Cutaneous Graft-versus-Host Disease: Observations in Four Patients

“…ECP sessions consisted of 2 treatment cycles on 2 successive days. Treatment frequency was standardized at 4-weekly intervals on the basis of our experience with patients suffering from scleroderma [11]using a technique already described [12]. On stabilization or improvement, treatment was gradually reduced depending on the clinical picture.…”

“…Manifestations of cGvHD were classified as either limited or extensive depending on the type of cutaneous involvement and the extent of systemic disease [11]. For diagnosis of limited cGvHD, skin lesions must exclusively be localized; no other organ except the liver must be affected.…”

Short-Time Extracorporeal Photochemotherapy in the Treatment of Drug-Resistant Autoimmune Bullous Diseases

“…Today, ECP is considered as one of the standard treatments for cutaneous T-cell lymphoma in the USA and several European countries [11]. Encouraging results in other T-cell-mediated disorders like chronic [12]and acute [13, 14]graft-versus-host disease as well as systemic [15, 16]and nodular [17]scleroderma (sclerosis) have been documented.…”