Cyclofenil was evaluated versus placebo in the treatment of
progressive systemic sclerosis (PSS, scleroderma) in a 2 x 6-month double-blind crossover study. The mean duration of disease was six years. Of 38 patients entering the study, 27 completed both periods. Reasons for drop-outs were very high liver
transaminases in three cases,
cardiac death in two, and
drug allergy, alcoholic problems, suspected
congestive heart failure, reactivation of
tuberculosis, arteriosclerotic
heart disease, and lethal progression of PSS in one case each. No fatality was attributed to
cyclofenil. Liver
enzyme abnormalities were seen in 13 of 35 active
drug periods and in 5 of 30 placebo periods. Cutaneous and visceral involvement were assessed by a large battery of subjective parameters and objective tests. Overall improvement was seen during 17
drug periods and nine placebo periods (N.S.), but a paired comparison of the status at the end of each treatment period resulted in the following distribution: 15 were improved at the end of the
drug period, four at the end of placebo period (p less than 0.01) and eight were unchanged. In patients with a disease duration of five years or less, joint stiffness and
pain were less on
drug than on placebo treatment (p less than 0.05). In the whole group, oesophageal peristalsis improved (p less than 0.05). Blood
folate increased (p less than 0.01). Working capacity was lower after the
drug period than after the placebo period (p less than 0.05). Several other parameters, however, did not change significantly.
Cyclofenil appears to be a promising
drug in the treatment of PSS and should be tested further in controlled long-term studies.