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.
Esta norma cita 3 dos 35 ensaios marcantes do nosso registo de Pneumologia.
Antifibrotic therapy2
- 2014INPULSISPneumo · DPI · Antifibrótico — FPIEm 1.066 doentes com fibrose pulmonar idiopática, o nintedanib 150 mg duas vezes ao dia abrandou o declínio anual da CVF em comparação com placebo.
- 2014ASCENDPneumo · DPI · Antifibrótico — FPIEm 555 doentes com FPI (CVF 50–90 % previsto), a pirfenidona 2.403 mg/dia reduziu o declínio da CVF ≥10 % ou morte às 52 semanas em comparação com placebo.
Against immunosuppression1
- 2012PANTHER-IPFPneumo · DPI · Imunossupressão — FPIEm doentes com fibrose pulmonar idiopática, a prednisona + azatioprina + NAC aumentou a mortalidade e a hospitalização em comparação com placebo.
Published since the guideline2
- 2019INBUILDPneumo · DPI · DPI fibrosante progressivaEm 663 doentes com DPI fibrosante progressiva não-FPI, o nintedanib abrandou o declínio anual da CVF em comparação com placebo.
- 2019SENSCISPneumo · DPI · DPI-SScEm 576 doentes com DPI associada a SSc, o nintedanib abrandou o declínio anual da CVF em comparação com placebo.