Idiopathic Pulmonary Fibrosis (IPF)
Patient Condition
The patient was 63 years old and developed post-exercise shortness of breath, which worsened progressively. The 64-year-old was diagnosed with idiopathic pulmonary fibrosis (IPF, type UIP) on HRCT and lung biopsy. After 2 years of treatment with nintedanib, lung function continued to decline (FVC decreased by approximately 350mL annually). On arrival, the 6-minute walking distance was 280m, aerobic saturation dropped to 82%, resting SpO was 92%, and HRCT showed extensive honeycomb lung and traction bronchiectasis.
Treatment Timeline
| Phase | Details | Date |
|---|---|---|
| Evaluation of joint anti-fibrosis program | ||
| Combination treatment with BMS-986278+ nintedanib | ||
| Maintenance treatment and functional maintenance |
Treatment Outcome
| before | Annual decline in FVC 350mL, 6MWD 280m, minimum SpO2 82%, extensive honeycomb lung |
|---|---|
| after | FVC decrease by 45mL/year (close to normal), 6MWD 430m, serum KL-6 halved, SGRQ improvement by 22 points |
| highlight | New LPA1 antagonist reduces the decline in pulmonary function in IPF by 87%, avoiding lung transplantation |