Colchicine, D-Penicillamine, and Prednisone in the Treatment of Idiopathic Pulmonary Fibrosis
A Controlled Clinical Trial
Idiopathic pulmonary fibrosis (IPF) is a chronic diffuse interstitial and intra-alveolar fibrosing inflammatory disease of unknown etiology that continues to represent an important therapeutic problem. Although the clinical course is variable, the disease is usually progressive and it is considered a highly lethal lung disease. Histopathologic findings include an inflammatory and a fibrotic component, with temporal and spatial inhomogeneity, and the cellular or the fibrotic predominance, when diagnosis is made, is considered the main prognostic factor.
Corticosteroids as well as other immunosuppressive or cytotoxic drugs are currently used with poor and transient response, or without success at all. Additionally, other pharmacologic approaches have been attempted, including colchicine, D-penicillamine, and cyclosporine, but most studies have been retrospective or uncontrolled trials Reading here buy birth control online. Particularly colchicine has created encouraging expectation, and a recent study comparing 22 patients who received colchicine with 22 historical patients who were given prednisone suggested that this drug may be of benefit in the treatment of IPF. Colchicine is an inhibitor of collagen synthesis and secretion, promotes collagenolytic activity, and suppresses the release of some fibroblast growth factors by alveolar macrophages; all of these putative antifibrotic properties support the notion that this drug could be useful as a therapeutic agent in lung fibrotic disorders.
D-penicillamine may inhibit collagen accumulation through disruption of several posttranscriptional steps of collagen synthesis, including crosslinking, and it has been used in fibrotic lung disease associated with progressive systemic sclerosis (PSS) with clinical and functional improvement. Interestingly, in the study of Steen et al, therapy with colchicine or immunosuppressive agents in PSS was not associated with similar improvement. However, this retrospective study has not been confirmed by a prospective, randomized study either in this disorder or in IPF.