Progressive systemic sclerosis -: treatment results of extracorporeal photopheresis

The treatment of progressive systemic sclerosis (PSS) is still unsatisfactory. We report on clinical,laboratory and immunological findings in 26 patients with PSS (6 males, 20 women) treated with extracorporeal photopheresis (ECP) for 8 cycles in a nonrandomized, uncontrolled study. ECP was performed on two consecutive days once a month. 8-methoxypsoralen concentrations in plasma and buffy coat were monitored by HPLC. We performed a standardized examination programme and determined parameters of inflammation and immune function. Global assessment revieled a partial remission in 18 patients,a stable disease in 8 patients and a slight progression in one patient. In the peripheral blood count a significant increase of CD3-positive NK cells was noted (p=0.03) although the leukocyte count decreased from 2,255 to 1,156 cells/mu l. There was a non-significant decrease of elastase (102.9 vs. 90.4 ng/ml), sulfidoleukotriens (2,255.4 vs. 1,688.9 pg/ml), ICAM-1 (301.9 vs. 276.6 ng/ml), soluble IL-2 receptor (609.0 vs. 422.3 U/ml), and IL-10 (164.7 vs. 138.7 pg/ml). IL-6 and IL-8 did not show significant changes. The ECP treatment of patients with PSS shows immunomodulatory effects changing levels of pro-inflammatory and cytokine substances. Even after 8 cycles partial remission or stable disease is seen in patients as shown by global assessment and certain clinical symptoms. On the other hand, sufficient data on the long-term outcome are still missing.