IPF: cyclophosphamide disappoints as adjunct for acute exacerbation

  • Hozumi H & al.
  • Respirology
  • 25 Feb 2019

  • curated by Jenny Blair, MD
  • Clinical Essentials
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Takeaway

  • 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.

Key results

  • Survival with vs without cyclophosphamide:
    • 90-day: 84.6% vs 76.9% (P=.70). 
    • Cumulative: no difference (P=.57).
    • Similar adverse-event rates.

Study design

  • 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.

Limitations

  • Potential residual confounders.
  • Not evaluated: cyclophosphamide with respect to long-term survival, steroid-sparing effect, or potential as salvage therapy.

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