PHOTOPHERESIS IN SYSTEMIC-SCLEROSIS - CLINICAL AND SEROLOGICAL STUDIES USING MARKERS OF COLLAGEN-METABOLISM

Eight patients with progressive systemic sclerosis were treated with p hotopheresis or extracorporeal photochemotherapy given on 2 consecutiv e days every 4 weeks for 5 to 8 months. Previous treatment with immuno suppressive agents or D-penicillamine was discontinued for at least 4 weeks prior to photopheresis. Although 11,2 receptor density in periph eral blood T-lymphocytes decreased significantly in the initial phase of the photopheresis therapy, no substantial clinical improvement occu rred. Although the intention had been to treat all patients for at lea st 8 months with photopheresis alone, it was mandatory due to severe e xacerbations to give additional immunotherapy to 4 patients, and 2 of these together with another patient wanted to discontinue photopheresi s after 5 and 6 months, as they did not expect an effect. Three of the 4 patients with progression had RNP-antibodies, suggesting that they had their scleroderma as part of a mixed connective tissue disease. Th e clinical exacerbations were accompanied in all patients by a highly significant increase in serum aminoterminal propeptide of type III pro collagen (PIIINP), which has been reported to correlate with involveme nt of skin and internal organs in systemic sclerosis. Similar but less significant increases were found in serum carboxyterminal propeptide of type I procollagen (PICP); there were no significant changes in ser um cross-linked fragment of type I collagen. Plasma levels of 8-methox ypsoralen were all above 80 ng/l, showing that the lack of responses t o photopheresis could not be due to poor absorption of the drug. Our d ata indicate that patients with the severe progressive form of systemi c sclerosis at least in a number of cases may not be sufficiently cont rolled by photopheresis alone but should be treated with immunosuppres sive agents. This may especially be the case if these patients have th eir scleroderma within the frame of a mixed connective tissue disease.