[Innovative therapeutics for idiopathic pulmonary fibrosis]

Abstract

Idiopathic pulmonary fibrosis (IPF), the most common and severe interstitial lung disease, remains a great challenge for clinicians. The natural history of the disease is incompletely understood and its prognosis is as devastating as that of many cancers. The most recent international consensus conference on IPF does not recommend any specific medical treatment and underlines the role of symptomatic care and management of co-morbidities, as well as lung transplantation, which should be openly discussed with the patient, if applicable, without delay. The lack of efficacy observed with steroids and immunosuppressive agents argues the current pathogenic hypothesis consistent with an abnormal alveolar epithelium wound healing after repeated injury. Thus, a need for anti-fibrosing and epithelial protective drugs emerged. Considerable progress has been made regarding the validation of relevant endpoints for clinical trials on IPF and pathogenesis, these two aspects of research being a framework for the exploration of new therapeutic approaches. Pirfenidone is the first drug that has been approved by health-care system for IPF treatment in Japan and in Europe. Many novel and promising drugs based on recent targets are under investigation. Combined therapies targeting different pathogenic pathways may represent the future for the treatment of IPF.