Ten patients with progressive, symptomatic
interstitial lung disease of unknown aetiology who were treated with
cyclosporin A were reviewed. Five had clinical and histopathological features of
cryptogenic fibrosing alveolitis and five a progressive restrictive
lung disease characterised by interstitial infiltration with lymphocytes and minimal
fibrosis, which could not be classified precisely. Three patients with lymphocytic infiltration showed a response to initial treatment with
cyclosporin A alone at high dosage, but toxicity precluded further treatment. All 10 patients then received low doses of
cyclosporin A and
prednisone. Three of the patients with
cryptogenic fibrosing alveolitis and all five patients with lymphocytic infiltration responded with a reduction in dyspnoea or an increase in vital capacity, or both;
cyclosporin A appeared to be effective, or at least to have a
corticosteroid potentiating effect. A high incidence of side effects occurred, though these do not necessarily prohibit the long term use of
cyclosporin A when it is indicated clinically.
Cyclosporin A may be effective in the treatment of
interstitial lung disease of unknown aetiology. Further studies are required to determine the long term outcome of treatment.