French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version - Archive ouverte HAL Access content directly
Journal Articles Respiratory Medicine and Research Year : 2023

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

1 IVPC - Infections Virales et Pathologie Comparée - UMR 754
2 CRMPM - Centre national de référence des maladies pulmonaires rares [Lyon]
3 LNC - Lipides - Nutrition - Cancer [Dijon - U1231]
4 CHU Dijon - Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand
5 Service de Pneumologie Soins Intensifs, Appareillage Respiratoire [CHU de Dijon]
6 CHU Tenon [AP-HP]
7 AP-HP - Hôpital Bichat - Claude Bernard [Paris]
8 Irset - Institut de recherche en santé, environnement et travail
9 EHESP - École des Hautes Études en Santé Publique [EHESP]
10 Hôpital Bretonneau
11 Hôpital Avicenne [AP-HP]
12 Institut Coeur Poumon [CHU Lille]
13 CHU Caen
14 CHU Pessac
15 PhyMedExp - Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046]
16 CIIL - Centre d’Infection et d’Immunité de Lille - INSERM U 1019 - UMR 9017 - UMR 8204
17 U933 - Maladies génétiques d'expression pédiatrique
18 CHRU Besançon - Centre Hospitalier Régional Universitaire de Besançon
19 Nouvel Hôpital Civil de Strasbourg
20 CHU Reims - Centre Hospitalier Universitaire de Reims
21 CHU - Hôpital Pasteur [Nice]
22 IRCAN - Institut de Recherche sur le Cancer et le Vieillissement
23 Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique
24 CHU Toulouse - Centre Hospitalier Universitaire de Toulouse
25 CHU - Centre Hospitalier Universitaire [Grenoble]
26 CHU Marseille
27 LITIS - Laboratoire d'Informatique, de Traitement de l'Information et des Systèmes
28 CIC Rouen - Centre d'Investigation Clinique [CHU Rouen]
29 HEGP - Hôpital Européen Georges Pompidou [APHP]
30 H&P - U1272 Inserm - Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires
31 CHRO - Centre Hospitalier Régional d'Orléans
32 HCL - Hospices Civils de Lyon
33 CH E.Muller Mulhouse - Centre Hospitalier Emile Muller [Mulhouse]
34 METRICS - Evaluation des technologies de santé et des pratiques médicales - ULR 2694
35 Centre Hospitalier Eure-Seine - Hôpital d'Evreux - Vernon (Evreux)
36 hpsj - Groupe Hospitalier Paris Saint-Joseph
37 Hôpital Louis Pradel - Services de Pneumologie, Exploration Fonctionnelle Respiratoire et Cardiologie
38 CREATIS - Centre de Recherche en Acquisition et Traitement de l'Image pour la Santé
Emmanuel Bergot
  • Function : Author
Elodie Blanchard
  • Function : Author
Emmanuel Gomez
  • Function : Author
Bruno Trumbic
  • Function : Author
Karim Berkani
  • Function : Author
Gérard Chatté
  • Function : Author
Jean-Michel Fourrier
  • Function : Author
Nicolas Just
  • Function : Author
Frédéric Le Guillou
  • Function : Author
Jean-Pierre Lhuillier
  • Function : Author
Philippe Terrioux
  • Function : Author

Abstract

BACKGROUND: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated. METHODS: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients’ association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale. RESULTS: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis. CONCLUSION: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis.

Dates and versions

hal-04087431 , version 1 (03-05-2023)

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Vincent Cottin, Philippe Bonniaud, Jacques Cadranel, Bruno Crestani, Stéphane Jouneau, et al.. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version. Respiratory Medicine and Research, 2023, 83, pp.100948. ⟨10.1016/j.resmer.2022.100948⟩. ⟨hal-04087431⟩
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