Background: Effective treatment modalities for systemic sclerosis, a life-t
hreatening and disabling disease, are still lacking. Possible efficacy of p
hotopheresis has been reported in several studies. Because of the complexit
y of the treatment, placebo-controlled trials are difficult to perform.
Objective: We investigated the effect of photopheresis on clinical paramete
rs (skin score and internal organ functions), immunologic parameters, and q
uality of life.
Methods: Nineteen patients with progressive systemic sclerosis of less than
5 years' duration were randomized into 2 groups. One group (group A) recei
ved photopheresis for 1 pear, the other group (group B) received no treatme
nt at all. After 1 year the groups switched (crossover design). Photopheres
is was performed on 2 consecutive days every 4 weeks; the psoralens were ad
ministered parenterally. The main outcome parameter was the skin score afte
r 1 year of treatment compared with that of the control group.
Results: The average skin score improved with 5.4% (standard error [SE], 20
.8%) in group A and deteriorated with 4.5% (SE, 13.8%) in group B (not sign
ificant; P = .71). Before crossover, the average increase in skin score was
5.3% (means of entire group). No change was observed in other clinical par
ameters. Approximately 1 year after crossover, the skin score reversed to w
hat would have been expected with an average increase of 5.3% per year. The
re was also no effect on immunologic parameters. Quality of life did not ch
ange during treatment.
Conclusion: We were not able to show that photopheresis, performed as descr
ibed above, is an effective treatment in systemic sclerosis. The difference
in average skin score was statistically and clinically insignificant. Desp
ite the small sample size, we concluded that the magnitude of the observed
changes is too small to justify photopheresis as a regular treatment.