Cyclophosphamide for Acute Exacerbation of Idiopathic Pulmonary Fibrosis
Status:
Completed
Trial end date:
2019-07-01
Target enrollment:
Participant gender:
Summary
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a major event of IPF with an
annual incidence between 5 and 10% and is responsible for the death of one third of IPF
patients. When AE-IPF occurs, it is associated with poor survival with an overall mortality
at 3 months upper of 50%. To date, no treatment has been proved to be effective in AE-IPF but
the efficacy of cyclophosphamide (CYC) on survival has been suggested, mainly by
retrospective series and needs to be confirmed. This confirmation is mandatory to improve
prognosis of AE-IPF but also to avoid unsuspected deleterious effect as it as been shown with
immunosuppressor in stable IPF.