An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline
The update that changed what idiopathic pulmonary fibrosis is treated with. Until 2011 the disease was managed as an inflammatory one, with prednisone, azathioprine and N-acetylcysteine given together; PANTHER-IPF stopped that arm early for death and hospitalisation, and this document recommends against the combination. Recommendations against warfarin, ambrisentan and imatinib follow the same logic: each had a plausible mechanism and a trial that found harm or nothing. What is left is antifibrotic: conditional recommendations for nintedanib and for pirfenidone, both of which slow the fall in forced vital capacity. Neither was shown to extend life in the trials the panel had, and the guideline is explicit that a conditional recommendation is what the evidence supports. The register carries the three trials that decided each of those questions, and INBUILD, which came later and extended nintedanib beyond IPF to progressive fibrosis of other causes.
Questa linea guida cita 3 dei 35 studi fondamentali del nostro registro di Pneumologia.
Antifibrotic therapy2
- 2014INPULSISPneumo · ILD · Antifibrotico — FPIIn 1.066 pazienti con fibrosi polmonare idiopatica, il nintedanib 150 mg due volte al giorno ha rallentato il declino annuale della FVC rispetto al placebo.
- 2014ASCENDPneumo · ILD · Antifibrotico — FPIIn 555 pazienti con FPI (FVC 50–90 % del predetto), la pirfenidone 2.403 mg/die ha ridotto il declino FVC ≥10 % o morte a 52 settimane rispetto al placebo.
Against immunosuppression1
- 2012PANTHER-IPFPneumo · ILD · Immunosoppressione — FPIIn pazienti con fibrosi polmonare idiopatica, prednisone + azatioprina + NAC ha aumentato la mortalità e l'ospedalizzazione rispetto al placebo.
Published since the guideline2
- 2019INBUILDPneumo · ILD · ILD progressivamente fibrosanteIn 663 pazienti con ILD progressivamente fibrosante non-FPI, il nintedanib ha rallentato il declino annuale della FVC rispetto al placebo.
- 2019SENSCISPneumo · ILD · SSc-ILDIn 576 pazienti con ILD associata a SSc, il nintedanib ha rallentato il declino annuale della FVC rispetto al placebo.