Therapeutic agent | Trial design | Drug combination | Conclusion | Reference |
ColchicineProposed effect: inhibits fibroblast collagen production and may reduce degradation354–356 | (1) Uncontrolled, randomised, prospective | Colchicine vs high-dose prednisolone | No survival advantage between groups | Douglas et al357 |
(2) Uncontrolled, non-randomised, prospective | Colchicine + prednisolone vs prednisolone only vs colchicine, penicillamine + prednisolone | Does not alter the course of steroid-treated IPF | Selman et al358 | |
PenicillamineProposed effect:inhibits collagen synthesis and cross linkage359 360 | (1) Uncontrolled, non-randomised, prospective | Penicillamine + prednisolone vs prednisolone only vs colchicine, penicillamine + prednisolone | Does not alter the course of steroid-treated IPF | Selman et al358 |
(2) Uncontrolled, non-randomised | Penicillamine + prednisolone vs azathioprine + prednisolone | No difference between groups | Meier-Sydow et al361 | |
CyclosporinProposed effect: suppresses T helper cells and TGFβ signalling362 363 | (1) Prospective, open, controlled | Cyclosporin | Numbers too few to analyse | Alton et al364 |
(2) Short-term, open label | Prednisolone + cyclosporine | Might permit reduction of steroids before transplantation | Venuta et al365 |
IPF, idiopathic pulmonary fibrosis; TGFβ, transforming growth factor β.