French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis – 2021 update. Full-length version - 06/06/23
Doi : 10.1016/j.resmer.2022.100948
Vincent Cottin a, ⁎ 
, Philippe Bonniaud b, Jacques Cadranel c, Bruno Crestani d, Stéphane Jouneau e, Sylvain Marchand-Adam f, Hilario Nunes g, Lidwine Wémeau-Stervinou h, Emmanuel Bergot i, Elodie Blanchard j, Raphaël Borie d, Arnaud Bourdin k, Cécile Chenivesse l, Annick Clément m, Emmanuel Gomez n, Anne Gondouin o, Sandrine Hirschi p, François Lebargy q, Charles-Hugo Marquette r, David Montani s, Grégoire Prévot t, Sébastien Quetant u, Martine Reynaud-Gaubert v, Mathieu Salaun w, Olivier Sanchez x, Bruno Trumbic y, Karim Berkani z, Pierre-Yves Brillet aa, Marion Campana ab, Lara Chalabreysse ac, Gérard Chatté ad, Didier Debieuvre ae, Gilbert Ferretti af, Jean-Michel Fourrier ag, Nicolas Just ah, Marianne Kambouchner ai, Bertrand Legrand aj, Frédéric Le Guillou ak, Jean-Pierre Lhuillier al, Anas Mehdaoui am, Jean-Marc Naccache an, Catherine Paganon ao, Martine Rémy-Jardin ap, Salim Si-Mohamed aq, Philippe Terrioux ar
and the
, Philippe Bonniaud b, Jacques Cadranel c, Bruno Crestani d, Stéphane Jouneau e, Sylvain Marchand-Adam f, Hilario Nunes g, Lidwine Wémeau-Stervinou h, Emmanuel Bergot i, Elodie Blanchard j, Raphaël Borie d, Arnaud Bourdin k, Cécile Chenivesse l, Annick Clément m, Emmanuel Gomez n, Anne Gondouin o, Sandrine Hirschi p, François Lebargy q, Charles-Hugo Marquette r, David Montani s, Grégoire Prévot t, Sébastien Quetant u, Martine Reynaud-Gaubert v, Mathieu Salaun w, Olivier Sanchez x, Bruno Trumbic y, Karim Berkani z, Pierre-Yves Brillet aa, Marion Campana ab, Lara Chalabreysse ac, Gérard Chatté ad, Didier Debieuvre ae, Gilbert Ferretti af, Jean-Michel Fourrier ag, Nicolas Just ah, Marianne Kambouchner ai, Bertrand Legrand aj, Frédéric Le Guillou ak, Jean-Pierre Lhuillier al, Anas Mehdaoui am, Jean-Marc Naccache an, Catherine Paganon ao, Martine Rémy-Jardin ap, Salim Si-Mohamed aq, Philippe Terrioux arand the
OrphaLung network
[a] National Coordinating Reference Center for Rare Lung Diseases, Pneumonology Department, Louis Pradel Hospital, Hospices Civils de Lyon (HCL), UMR 754, IVPC, INRAE, Member of OrphaLung, RespiFil, Radico-ILD2 and ERN-LUNG, Lyon University, Claude Bernard Lyon 1 University, 28 avenue Doyen Lépine, Lyon F-69677 CEDEX, France
[b] Constitutive Reference Center for Rare Lung Diseases, Bourgogne University Hospital Center and Faculty of Medicine and Pharmacy, Bourgogne-Franche Comté University, INSERM U123-1, Dijon, France
[c] Constitutive Reference Center for Rare Lung Diseases, Assistance Publique-Hôpitaux de Paris (AP-HP), Tenon Hospital, Sorbonne University, GRC 04 Theranoscan, Paris, France
[d] Constitutive Reference Center for Rare Lung Diseases, Pneumonology Department A, AP-HP, Bichat Hospital, Paris, France
[e] Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, Pontchaillou Hospital, IRSET UMR1085, Rennes 1 University, Rennes, France
[f] Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, CHRU, Bretonneau Hospital, Tours, France
[g] Constitutive Reference Center for Rare Lung Diseases, Pneumonology Department, Avicenne Hospital, Bobigny, AP-HP, Sorbonne Paris Nord University, Bobigny, France
[h] Constitutive Reference Center for Rare Lung Diseases, Heart-Lung Institute, Pneumonology and Immuno-allergology Department, CHRU of Lille, Lille, France
[i] Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, Pneumonology and Thoracic Oncology Department, Côte de Nacre Hospital, Caen University Hospital Center, Caen, France
[j] Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, Haut Levêque Hospital, Bordeaux University Hospital Center, Pessac, France
[k] Expert Center for Rare Lung Diseases in Adults, Pneumonology and Addictology Department, INSERM U1046, CNRS UMR 921, Arnaud de Villeneuve Hospital, Montpellier University Hospital Center, Montpellier, France
[l] Constitutive Reference Center for Rare Lung Diseases, Pneumonology and Immuno-allergology Department, Albert Calmette Hospital, Lille Regional University Hospital Center, Infection and Immunity Center of Lille U1019 - UMR 9017, Lille University, Lille University Hospital Center, CNRS, Inserm, Pasteur Institute of Lille, Lille, France
[m] 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, Sorbonne University, Paris, France
[n] Expert Center for Rare Lung Diseases, Pneumonology Department, Brabois Hospitals, Nancy Regional University Hospital Center, Vandoeuvre-les Nancy, France
[o] Expert Center for Rare Lung Diseases, Pneumonology Department, HP, Jean Minjoz Univerty Hospital Center, Besançon, France
[p] Expert Center for Rare Lung Diseases, Pneumonology Department, New Civil Hospital, Strasbourg, France
[q] Expert Center for Rare Lung Diseases, Pneumonology Department, Maison Blanche University Hospital Center, Reims, France
[r] Expert Center for Rare Lung Diseases, FHU OncoAge, Nice cedex 1, Pneumonology and Thoracic Oncology Department, Institute of Research on Cancer and Aging (IRCAN), Pasteur Hospital, Nice University Hospital Center, Côte d'Azur University, CNRS, INSERM, Nice, France
[s] Expert Center for Rare Lung Diseases, National Coordinating Reference Center for Pulmonary Hypertension, Pneumonology and Pneumological Intensive Care Unit, DMU 5 Thorinno, Inserm UMR S999, Faculty of Medicine, AP-HP, Paris-Sud University Hospital Center, Bicêtre Hospital, Paris-Saclay University, Le Kremlin-Bicêtre, France
[t] Expert Center for Rare Lung Diseases, Pneumonology Department, Larrey University Hospital Center, Toulouse, France
[u] Expert Center for Rare Lung Diseases, Pneumonology and Physiology Department, Grenoble Alpes University Hospital Center, Grenoble, France
[v] 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, France
[w] Expert Center for Rare Lung Diseases, Pneumonology, Thoracic Oncology and Respiratory Intensive Care Department & CIC 1404, Charles Nicole Hospital, Rouen University Hospital Center, IRIB, QuantiIF-LITIS Laboratory, EA 4108, Rouen University, Rouen, France
[x] Expert Center for Rare Lung Diseases, Pneumonology and Intensive Care Department, Georges Pompidou European Hospital, AP-HP, Paris, France
[y] Cap Evidence, Paris, France
[z] Pierre de Soleil Clinic, Vetraz Monthoux, France
[aa] UPRES EA 2363, Radiology Department, AP-HP, Avicenne Hospital, Paris 13 University, Bobigny, France
[ab] Pneumonology and Thoracic Oncology Department, Orléans Regional Hospital Center, Orléans, France
[ac] Pathological Anatomy Department, East Hospital Group, HCL, Bron, France
[ad] Pneumonology Practice and Protestant Infirmary, Caluire, France
[ae] Pneumonology Department, GHRMSA, Emile Muller Hospital, Mulhouse, France
[af] Diagnostic and Interventional Radiology Department, Grenoble Alpes University Hospital Center, Grenoble Alpes University, Grenoble, France
[ag] Pierre Enjalran Association for Idiopathic Pulmonary Fibrosis (APEFPI), Meyzieu, France
[ah] Pneumonology Department, Victor Provo Hospital Center, Roubaix, France
[ai] Pathology Department, AP-HP, Avicenne Hospital, Bobigny, France
[aj] Medical Practice of Bourgogne, ULR 2694 METRICS, CERIM, Lille University, Lille University Hospital Center, Tourcoing, Lille, France
[ak] Pneumonology Practice, Esquirol Health Pole, Le Pradet, France
[al] Pneumonology Practice, La Varenne Saint-Hilaire, France
[am] Pneumonology and Thoracic Oncology Department, Eure-Seine Hospital Center, Évreux, France
[an] Pneumonology, Allergology and Thoracic Oncology Department, Paris Saint-Joseph Hospital Group, Paris, France
[ao] Pneumonology Department, Louis Pradel Hospital, HCL, Bron, France
[ap] Heart-Lung Institute, Radiology and Thoracic Imaging Department, Lille Regional University Hospital Center, Lille, France
[aq] Cardiovascular and Thoracic Imaging Department, Louis Pradel Hospital, HCL, INSA-Lyon, UJM-Saint Etienne, CNRS, Inserm, CREATIS UMR 5220, Lyon University, Claude Bernard Lyon 1 University, U1206, Villeurbanne, France
[ar] Pneumonology Practice, Meaux, France
⁎Corresponding author.
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 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.
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.
Vol 83
Article 100948- juin 2023 Retour au numéro
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