- For patients with idiopathic pulmonary fibrosis (IPF) experiencing acute exacerbation (AE), adding cyclophosphamide to corticosteroid treatment does not improve survival.
Why this matters
- After AE, death typically occurs within a few months.
- These patients are typically treated with high-dose corticosteroids.
- Cyclophosphamide has been tried in uncontrolled studies.
- Survival with vs without cyclophosphamide:
- 90-day: 84.6% vs 76.9% (P=.70).
- Cumulative: no difference (P=.57).
- Similar adverse-event rates.
- Retrospective study using propensity matching at 3 hospitals in Japan (n=102).
- Participants: consecutive patients with idiopathic AE of IPF.
- Authors compared 26 matched pairs after corticosteroid treatment with vs without cyclophosphamide.
- Outcomes: post-AE 90-day survival, post-AE cumulative survival, treatment safety.
- Funding: None disclosed.
- Potential residual confounders.
- Not evaluated: cyclophosphamide with respect to long-term survival, steroid-sparing effect, or potential as salvage therapy.