Idiopathic pulmonary fibrosis
FDA approves nerandomilast for treatment of idiopathic pulmonary fibrosis in adults
Idiopathic pulmonary fibrosis (IPF) has few treatment options and currently relies on antifibrotic agents (nintedanib and pirfenidone) and supportive therapy. Nerandomilast, an oral phosphodiesterase-4 (PDE-4) inhibitor, is the first new treatment for IPF approved by the Food and Drug Administration (FDA) in more than 10 years.
One randomized, double-blind, placebo-controlled phase 3 trial demonstrated that people with IPF taking nerandomilast had a significantly smaller decline in forced vital capacity (FVC) compared with placebo-treated groups over a period of 1 year.[84]
Nerandomilast can be used alone or in combination with antifibrotic therapy.
This treatment has the potential to impact the care of many patients, including those with:
Intolerance to an antifibrotic agent.
Continued worsening in lung function despite use of an antifibrotic agent.
Summary
Definition
Anamnesis y examen
Principales factores de diagnóstico
- dyspnea
- cough
- crackles
Otros factores de diagnóstico
- weight loss, fatigue, and malaise
- clubbing
Factores de riesgo
- advanced age
- male sex
- family history
- gene mutations and nucleotide polymorphisms
- cigarette smoking
- occupational and environmental exposures
- gastroesophageal reflux
- viral infection
- bacterial infection
- diabetes
Pruebas diagnósticas
Primeras pruebas diagnósticas para solicitar
- CXR
- high-resolution CT (HRCT) chest
- pulmonary function tests
Pruebas diagnósticas que deben considerarse
- surgical lung biopsy
- bronchoalveolar lavage (BAL)
- transbronchial lung biopsy and cryobiopsy
- CRP
- erythrocyte sedimentation rate (ESR)
- antinuclear antibody immunofluorescence
- rheumatoid factor
- anticyclic citrullinated peptide
- myositis panel
Algoritmo de tratamiento
acute exacerbation
not currently experiencing acute exacerbation
Colaboradores
Autores
Jake G. Natalini, MD, MS
Assistant Professor of Medicine
Pulmonary, Critical Care, and Sleep Division
NYU Grossman School of Medicine
New York University
New York
NY
Divulgaciones
JGN has received consulting fees from CareDx on diagnostics in lung transplantation, not related to the article topic. There is not a contractual agreement to disseminate product information.
Agradecimientos
Dr Jake G. Natalini would like to gratefully acknowledge Dr Mary Elizabeth Kreider, Dr Judd David Flesch, Dr Gregory Tino, and Dr Jeffrey C. Munson, previous contributors to this topic.
Divulgaciones
GT has served as a consultant for InterMune. GT has served as a principal investigator for clinical trials in idiopathic pulmonary fibrosis. JCM and JDF declare that they have no competing interests.
Revisores por pares
Stephen Nathan, MD
Medical Director
Lung Transplant & Advanced Lung Disease Program
Inova Fairfax Hospital
Falls Church
VA
Divulgaciones
SN is an author of a reference cited in this monograph.
Athol Wells, MD, FRCP
Professor of Respiratory Medicine
Interstitial Lung Disease Unit
Royal Brompton Hospital
London
UK
Divulgaciones
AW declares that he has no competing interests.
Agradecimiento de los revisores por pares
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Divulgaciones
Las afiliaciones y divulgaciones de los revisores por pares se refieren al momento de la revisión.
Referencias
Artículos principales
Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis - an update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015 Jul 15;192(2):e3-19.Texto completo Resumen
Raghu G, Remy-Jardin M, Richeldi L, et al; American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2022 May 1;205(9):e18-e47.Texto completo Resumen
Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. an international working group report. Am J Respir Crit Care Med. 2016 Aug 1;194(3):265-75.Texto completo Resumen
Borie R, Kannengiesser C, Antoniou K, et al. European Respiratory Society statement on familial pulmonary fibrosis. Eur Respir J. 2023 Mar;61(3):2201383.Texto completo Resumen
Ryerson CJ, Adegunsoye A, Piciucchi S, et al. Update of the international multidisciplinary classification of the interstitial pneumonias: an ERS/ATS statement. Eur Respir J. 2025 Dec;66(6):2500158.Texto completo Resumen
Artículos de referencia
Una lista completa de las fuentes a las que se hace referencia en este tema está disponible para los usuarios con acceso a todo BMJ Best Practice.
Diferenciales
- Nonspecific interstitial pneumonia (NSIP)
- Bronchiolocentric interstitial pneumonia (BIP)
- Organizing pneumonia
Más DiferencialesGuías de práctica clínica
- Update of the international multidisciplinary classification of the interstitial pneumonias: an ERS/ATS statement
- ERS statement on transition of care in childhood interstitial lung diseases
Más Guías de práctica clínicaVideos
Late inspiratory crackles (rales)
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