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French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis - 2021 update. Full-length version - 04/08/22

Doi : 10.1016/j.resmer.2022.100948 
Vincent Cottin 1, , Philippe Bonniaud 2, Jacques Cadranel 3, Bruno Crestani 4, Stéphane Jouneau 5, Sylvain Marchand-Adam 6, Hilario Nunes 7, Lidwine Wémeau-Stervinou 8, Emmanuel Bergot 9, Elodie Blanchard 10, Raphaël Borie 4, Arnaud Bourdin 11, Cécile Chenivesse 12, Annick Clément 13, Emmanuel Gomez 14, Anne Gondouin 15, Sandrine Hirschi 16, François Lebargy 17, Charles-Hugo Marquette 18, David Montani 19, Grégoire Prévot 20, Sébastien Quetant 21, Martine Reynaud-Gaubert 22, Mathieu Salaun 23, Olivier Sanchez 24, Bruno Trumbic 25, Karim Berkani 26, Pierre-Yves Brillet 27, Marion Campana 28, Lara Chalabreysse 29, Gérard Chatté 30, Didier Debieuvre 31, Gilbert Ferretti 32, Jean-Michel Fourrier 33, Nicolas Just 34, Marianne Kambouchner 35, Bertrand Legrand 36, Frédéric Le Guillou 37, Jean-Pierre Lhuillier 38, Anas Mehdaoui 39, Jean-Marc Naccache 40, Catherine Paganon 41, Martine Rémy-Jardin 42, Salim Si-Mohamed 43, Philippe Terrioux 44
1 National Coordinating Reference Center for Rare Lung Diseases, Pneumonology Department, Louis Pradel Hospital, Hospices Civils de Lyon (HCL), Bron; UMR 754, IVPC, INRAE, Lyon University, Claude Bernard Lyon 1 University, Lyon; member of OrphaLung, RespiFil, Radico-ILD2 and ERN-LUNG, Lyon 
2 Constitutive Reference Center for Rare Lung Diseases, Bourgogne University Hospital Center and Faculty of Medicine and Pharmacy, Bourgogne-Franche Comté University, Dijon; INSERM U123-1, Dijon 
3 Constitutive Reference Center for Rare Lung Diseases, Assistance Publique-Hôpitaux de Paris (AP-HP), Tenon Hospital, Paris; Sorbonne University GRC 04 Theranoscan, Paris 
4 Constitutive Reference Center for Rare Lung Diseases, Pneumonology Department A, AP-HP, Bichat Hospital, Paris 
5 Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, Pontchaillou Hospital, Rennes; IRSET UMR1085, Rennes 1 University, Rennes 
6 Expert Center for Rare Lung Diseases in Adults, Bretonneau Hospital, Pneumonology Department, CHRU, Tours 
7 Constitutive Reference Center for Rare Lung Diseases, Pneumonology Department, AP-HP, Avicenne Hospital, Bobigny; Sorbonne Paris Nord University, Bobigny 
8 Constitutive Reference Center for Rare Lung Diseases, Heart-Lung Institute, Pneumonology and Immuno-allergology Department, CHRU of Lille, Lille 
9 Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, Pneumonology and Thoracic Oncology Department, Côte de Nacre Hospital, Caen University Hospital Center, Caen 
10 Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, Haut Levêque Hospital, Bordeaux University Hospital Center, Pessac 
11 Expert Center for Rare Lung Diseases in Adults, Pneumonology and Addictology Department, Arnaud de Villeneuve Hospital, Montpellier University Hospital Center, Montpellier; INSERM U1046, CNRS UMR 921, Montpellier 
12 Constitutive Reference Center for Rare Lung Diseases, Pneumonology and Immuno-allergology Department, Albert Calmette Hospital; Lille Regional University Hospital Center, Lille; Infection and Immunity Center of Lille U1019 - UMR 9017, Lille University, Lille University Hospital Center, CNRS, Inserm, Pasteur Institute of Lille, Lille 
13 Resource and Expert Center for Pediatric Cystic Fibrosis, Reference Center for Rare Respiratory Diseases (RespiRare), Pediatric Pneumonology Department, Armand Trousseau Children's Hospital, Paris Est University Hospital Center, Paris; Sorbonne University, Paris 
14 Expert Center for Rare Lung Diseases, Pneumonology Department, Brabois Hospitals, Nancy Regional University Hospital Center, Vandoeuvre-les Nancy 
15 Expert Center for Rare Lung Diseases, Pneumonology Department, HP, Jean Minjoz Univerty Hospital Center, Besançon 
16 Expert Center for Rare Lung Diseases, Pneumonology Department, New Civil Hospital, Strasbourg 
17 Expert Center for Rare Lung Diseases, Pneumonology Department, Maison Blanche University Hospital Center, Reims 
18 Expert Center for Rare Lung Diseases, FHU OncoAge, Pneumonology and Thoracic Oncology Department, Pasteur Hospital, Nice University Hospital Center, Nice cedex 1; Côte d'Azur University, CNRS, INSERM, Institute of Research on Cancer and Aging (IRCAN), Nice, 
19 Expert Center for Rare Lung Diseases, National Coordinating Reference Center for Pulmonary Hypertension, Pneumonology and Pneumological Intensive Care Unit, AP-HP, DMU 5 Thorinno, Inserm UMR S999, Paris-Sud University Hospital Center, Bicêtre Hospital, Le Kremlin-Bicêtre; Paris-Saclay University, Faculty of Medicine, Le Kremlin-Bicêtre 
20 Expert Center for Rare Lung Diseases, Pneumonology Department, Larrey University Hospital Center, Toulouse 
21 Expert Center for Rare Lung Diseases, Pneumonology and Physiology Department, Grenoble Alpes University Hospital Center, Grenoble. 
22 Expert Center for Rare Lung Diseases, Pneumonology Department, AP-HM, Nord University Hospital Center, Marseille; Aix Marseille University, IRD, APHM, MEPHI, IHU-Méditerranée Infection, Marseille 
23 Expert Center for Rare Lung Diseases, Pneumonology, Thoracic Oncology and Respiratory Intensive Care Department & CIC 1404, Charles Nicole Hospital, Rouen, Rouen University Hospital Center; IRIB, QuantiIF-LITIS laboratory, EA 4108, Rouen University 
24 Expert Center for Rare Lung Diseases, Pneumonology and Intensive Care Department, Georges Pompidou European Hospital, AP-HP, Paris 
25 Cap Evidence, Paris 
26 Pierre de Soleil Clinic, Vetraz Monthoux 
27 Paris 13 University, UPRES EA 2363, Bobigny; Radiology Department, AP-HP, Avicenne Hospital, Bobigny 
28 Pneumonology and Thoracic Oncology Department, Orléans Regional Hospital Center, Orléans 
29 Pathological Anatomy Department, East Hospital Group, HCL, Bron. 
30 Pneumonology Practice and Protestant Infirmary, Caluire 
31 Pneumonology Department, GHRMSA, Emile Muller Hospital, Mulhouse 
32 Grenoble Alpes University, Grenoble; Diagnostic and Interventional Radiology Department, Grenoble Alpes University Hospital Center, Grenoble 
33 Pierre Enjalran Association for Idiopathic Pulmonary Fibrosis (APEFPI), Meyzieu 
34 Pneumonology Department, Victor Provo Hospital Center, Roubaix 
35 Pathology Department, AP-HP, Avicenne Hospital, Bobigny 
36 Medical Practice of Bourgogne, Tourcoing; Lille University, Lille University Hospital Center, ULR 2694 METRICS, CERIM, Lille. 
37 Pneumonology Practice, Esquirol Health Pole, Le Pradet 
38 Pneumonology Practice, La Varenne Saint-Hilaire 
39 Pneumonology and Thoracic Oncology Department, Eure-Seine Hospital Center, Évreux 
40 Pneumonology, Allergology and Thoracic Oncology Department, Paris Saint-Joseph Hospital Group, Paris 
41 Pneumonology Department, Louis Pradel Hospital, HCL, Bron 
42 Heart-Lung Institute, Radiology and Thoracic Imaging Department, Lille Regional University Hospital Center, Lille 
43 Cardiovascular and Thoracic Imaging Department, Louis Pradel Hospital, HCL, Bron; Lyon University, INSA-Lyon, Claude Bernard Lyon 1 University, UJM-Saint Etienne, CNRS, Inserm, CREATIS UMR 5220, U1206, Villeurbanne 
44 Pneumonology Practice, Meaux 

Correspondence: Vincent Cottin, Centre national de référence coordonnateur des maladies pulmonaires rares, Hôpital Louis Pradel, Hospices civils de Lyon, Bâtiment A4 Pneumologie, Groupement Hospitalier Est, 28 avenue Doyen Lépine, F-69677 Lyon cedexCentre national de référence coordonnateur des maladies pulmonaires raresHôpital Louis Pradel, Hospices civils de Lyon, Bâtiment A4 Pneumologie, Groupement Hospitalier Est28 avenue Doyen LépineLyon cedexF-69677
Sous presse. Manuscrit accepté. Disponible en ligne depuis le Thursday 04 August 2022

Abstract

Background

Since the latest 2017 French guidelines, knowledge about idiopathic pulmonary fibrosis has evolved considerably.

Methods

Practical guidelines were drafted on the initiative of the Coordinating Reference Center for Rare Lung 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.

Results

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.

Conclusion

These evidence-based guidelines are intended to guide the diagnosis and practical management of idiopathic pulmonary fibrosis.

Le texte complet de cet article est disponible en PDF.

Keywords : Pulmonary fibrosis, Interstitial lung disease, Biopsy, Usual interstitial pneumonia


Plan


 Funding and support: Association pour l'amélioration des maladies pulmonaires rares; Hospices Civils de Lyon; Université Claude Bernard Lyon 1.


© 2022  Publié par Elsevier Masson SAS.
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