French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version

Since the latest 2017 French guidelines, knowledge about idiopathic pulmonary fibrosis has evolved considerably. Practical guidelines were drafted on the initiative of the Coordinating Reference Center for Rare Pulmonary Diseases, led by the French Language Pulmonology Society (SPLF), by a coordinat...

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Published in:Respiratory medicine and research 2023-06, Vol.83, p.100948-100948, Article 100948
Main Authors: Cottin, Vincent, Bonniaud, Philippe, Cadranel, Jacques, Crestani, Bruno, Jouneau, Stéphane, Marchand-Adam, Sylvain, Nunes, Hilario, Wémeau-Stervinou, Lidwine, Bergot, Emmanuel, Blanchard, Elodie, Borie, Raphaël, Bourdin, Arnaud, Chenivesse, Cécile, Clément, Annick, Gomez, Emmanuel, Gondouin, Anne, Hirschi, Sandrine, Lebargy, François, Marquette, Charles-Hugo, Montani, David, Prévot, Grégoire, Quetant, Sébastien, Reynaud-Gaubert, Martine, Salaun, Mathieu, Sanchez, Olivier, Trumbic, Bruno, Berkani, Karim, Brillet, Pierre-Yves, Campana, Marion, Chalabreysse, Lara, Chatté, Gérard, Debieuvre, Didier, Ferretti, Gilbert, Fourrier, Jean-Michel, Just, Nicolas, Kambouchner, Marianne, Legrand, Bertrand, Le Guillou, Frédéric, Lhuillier, Jean-Pierre, Mehdaoui, Anas, Naccache, Jean-Marc, Paganon, Catherine, Rémy-Jardin, Martine, Si-Mohamed, Salim, Terrioux, Philippe
Format: Article
Language:English
Subjects:
Biopsy
Human health and pathology
Humans
Idiopathic Pulmonary Fibrosis - diagnosis
Idiopathic Pulmonary Fibrosis - epidemiology
Idiopathic Pulmonary Fibrosis - therapy
Interstitial lung disease
Life Sciences
Lung - pathology
Lung Transplantation
Prognosis
Pulmonary fibrosis
Pulmonology and respiratory tract
Santé publique et épidémiologie
Tomography, X-Ray Computed - methods
Usual interstitial pneumonia
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Summary:Since the latest 2017 French guidelines, knowledge about idiopathic pulmonary fibrosis has evolved considerably. Practical guidelines were drafted on the initiative of the Coordinating Reference Center for Rare Pulmonary Diseases, led by the French Language Pulmonology Society (SPLF), by a coordinating group, a writing group, and a review group, with the involvement of the entire OrphaLung network, pulmonologists practicing in various settings, radiologists, pathologists, a general practitioner, a health manager, and a patient association. The method followed the "Clinical Practice Guidelines" process of the French National Authority for Health (HAS), including an online vote using a Likert scale. After a literature review, 54 guidelines were formulated, improved, and then validated by the working groups. These guidelines addressed multiple aspects of the disease: epidemiology, diagnostic procedures, quality criteria and interpretation of chest CT scans, lung biopsy indication and procedures, etiological workup, methods and indications for family screening and genetic testing, assessment of the functional impairment and prognosis, indication and use of antifibrotic agents, lung transplantation, management of symptoms, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis. These evidence-based guidelines are intended to guide the diagnosis and practical management of idiopathic pulmonary fibrosis.
ISSN:2590-0412
2590-0412
DOI:10.1016/j.resmer.2022.100948