Fibrose pulmonar idiopática
A FDA aprova o nerandomilast para o tratamento da fibrose pulmonar idiopática em adultos
A fibrose pulmonar idiopática (FPI) apresenta poucas opções de tratamento e atualmente depende de agentes antifibróticos (nintedanibe e pirfenidona) e terapia de suporte. O nerandomilast, um inibidor oral da fosfodiesterase-4 (PDE-4), é o primeiro novo tratamento para a FPI aprovado pela Food and Drug Administration (FDA) dos EUA em mais de 10 anos.
Um ensaio clínico randomizado, duplo-cego e controlado por placebo de fase 3 demonstrou que pessoas com FPI que tomaram nerandomilast apresentaram uma diminuição significativamente menor na capacidade vital forçada (CVF) em comparação com os grupos tratados com placebo ao longo de um período de 1 ano.[107] O nerandomilast pode ser usado isoladamente ou em combinação com terapia antifibrótica.
Este tratamento tem o potencial de impactar o atendimento de muitos pacientes, incluindo aqueles com:
intolerância a um agente antifibrótico e
agravamento contínuo da função pulmonar, apesar do uso de um agente antifibrótico.
Summary
Definition
History and exam
Key diagnostic factors
- presença de fatores de risco
- dispneia
- tosse
- estertores
Other diagnostic factors
- perda de peso, fadiga e mal-estar
- baqueteamento digital
Risk factors
- idade avançada
- sexo masculino
- história familiar
- mutações genéticas e polimorfismos de nucleotídeos
- tabagismo
- exposições ocupacionais e ambientais
- refluxo gastroesofágico
- infecção viral
- infecção bacteriana
- diabetes
Diagnostic tests
1st tests to order
- radiografia torácica
- TC de alta resolução (TCAR) do tórax
- testes de função pulmonar
Tests to consider
- biópsia pulmonar cirúrgica
- lavagem broncoalveolar (LBA)
- biópsia pulmonar e criobiópsia transbrônquica
- proteína C-reativa
- velocidade de hemossedimentação (VHS)
- imunofluorescência para fator antinuclear
- fator reumatoide
- antipeptídeo citrulinado cíclico
- perfil de miosite
Treatment algorithm
exacerbação aguda
todos os pacientes sem exacerbação aguda no momento
Contributors
Authors
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
Disclosures
JGN is serving as site principal investigator for a CareDx clinical diagnostics study in lung transplantation. His institution receives payments for this trial. He has also 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 for CareDx.
Acknowledgements
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.
Disclosures
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.
Peer reviewers
Stephen Nathan, MD
Medical Director
Lung Transplant & Advanced Lung Disease Program
Inova Fairfax Hospital
Falls Church
VA
Disclosures
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
Disclosures
AW declares that he has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824.Full text Abstract
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.Full text Abstract
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.Full text Abstract
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.Full text Abstract
Borie R, Kannengiesser C, Antoniou K, et al. European Respiratory Society statement on familial pulmonary fibrosis. Eur Respir J. 2023 Mar;61(3):2201383.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Pneumonia intersticial idiopática não específica
- Pneumonia em organização criptogênica (anteriormente conhecida como bronquiolite obliterante com pneumonia em organização [BOPO])
- Pneumonia intersticial aguda (PIA)
More DifferentialsGuidelines
- European Respiratory Society clinical practice guideline on symptom management for adults with serious respiratory illness
- ERS statement on transition of care in childhood interstitial lung diseases
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