Management of Progressive Disease in Idiopathic Pulmonary Fibrosis
Status:
Recruiting
Trial end date:
2022-12-01
Target enrollment:
Participant gender:
Summary
Idiopathic pulmonary fibrosis (IPF) is a prototype of chronic, progressive, and fibrotic lung
disease. It has been considered rare, with an incidence estimated to 11.5 cases per 100 000
individuals per year. Increasing rates of hospital admissions and deaths due to IPF suggest
an increasing burden of disease. The median survival time from diagnosis is 2-4 years.
Recently two disease-modifying therapies, pirfenidone and nintedanib, have been approved
worldwide. Both drugs reduce the disease progression as measured by progressive decline in
forced vital capacity (FVC), with a reduction of overall mortality showed by meta-analysis of
phase III pirfenidone trials.
However, progression of disease continues to occur despite the currently available drug
therapy. Many patients die from progressive, chronic hypoxemic respiratory failure, or less
frequently from acute exacerbation of pulmonary fibrosis. In these patients, no data are
available to guide management between continuation of the prescribed antifibrotic drug, to
switch to the other available antifibrotic drug, or to combine the available drugs.
The combination of nintedanib and pirfenidone is not recommended outside clinical trials.
However, although both antifibrotic drugs were developed and approved as monotherapy, two
recent trials have suggested the feasibility and safety of combining them over a 12-24 weeks
period. These results encourage further studies of combination treatment with pirfenidone and
nintedanib in patients with IPF. Such study is timely, as there is a risk that clinicians
facing the continued worsening of disease in patients receiving one of the available drugs
may prescribe both drugs combined outside clinical trials, potentially exposing patients to a
currently unknown risk.
This study will evaluate the efficacy and tolerance of the combination pirfenidone and
nintedanib as compared to a "switch monotherapy": i.e. switching from the current to the
other of the two existing drugs prescribed as monotherapy, in patients who present chronic
worsening IPF despite receiving either pirfenidone or nintedanib and as to a "control group":
i.e.treatment still be on as before randomization (pirfenidone or nintedanib).