Background. Cyclosporin and tacrolimus are immunomodulatory drugs which act
predominantly on T cells. Improvements in certain manifestations, particul
arly skin tightness, have been observed in a number of patients with sclero
derma treated with these drugs. However, to date there have been no reports
of their use in a routine clinical setting.
Methods. Patients attending clinical immunology clinics who had progressive
systemic sclerosis and related syndromes and who had received cyclosporin
and/or tacrolimus were identified. Details of their treatment, including dr
ug dosage, duration of and response to treatment, side-effects and reasons
for withdrawal, were recorded.
Results. Sixteen patients had been given cyclosporin and 13 of these had be
en treated for skin tightness. Half noticed significant softening of their
skin whilst on treatment, and resolution was observed in all four of the pa
tients treated for digital vasculitis. Side-effects were common and dose-li
miting, and contributed to withdrawal in 12 out of 13 patients. Eight patie
nts had been treated with tacrolimus; two of these had stopped the drug bec
ause of progression of their disease, one developed diarrhoea, prompting wi
thdrawal, one stopped tacrolimus following improvement, and four remained o
n the drug. Side-effects had occurred in three patients.
Conclusion. Improvements in skin occur in approximately half of all cases o
f scleroderma treated with either cyclosporin or tacrolimus, suggesting a b
eneficial effect. Side-effects, especially hypertension, are common with cy
closporin and often necessitate withdrawal. Adverse effects are also observ
ed with tacrolimus, but in the small cohort so far treated only one patient
had stopped the drug for this reason.