MANAGEMENT OF SEVERE SCLERODERMA WITH LONG-TERM EXTRACORPOREAL PHOTOPHERESIS

Background: The management of systemic sclerosis remains unsatisfactor y. Thus far, the action of extracorporeal photopheresis (ECP) in sever e systemic scleroderma has been evaluated in short-term studies, and o nly limited experience has been obtained with long-term application. O bjective: The aim of the present study was to evaluate prospectively t he long-term effect of ECP in a group of 16 patients suffering from se vere scleroderma, showing visceral involvement and progressive clinica l course. Methods: Fourteen patients with systemic scleroderma involvi ng 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, gamma-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. Re sults: 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 wi th ECP early after onset of scleroderma (less than or equal to 2 years ), whereas all patients with a progressive course under ECP had had sc leroderma for longer than 2 years. Immunosuppressive drugs previously administered could be reduced or fully withdrawn under ECP treatment i n 5 patients, bur additional oral medication was introduced in 4 patie nts due to disease progression. Addition of gamma-IFN to ECP did not r eveal further benefit. No side-effects were recorded under ECP treatme nt. Conclusions: Based on this observation, we believe that long-term ECP represents an effective treatment modality in severe scleroderma p articularly when started early, with stabilization of the disease cour se and partial remission of the cutaneous findings, whereas visceral i nvolvement, if present, may rarely improve.