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

Vincent Cottin; Philippe Bonniaud; Jacques Cadranel; Bruno Crestani; Stéphane Jouneau; Sylvain Marchand-Adam; Hilario Nunes; Lidwine Wémeau-Stervinou; Emmanuel Bergot; Elodie Blanchard; Raphaël Borie; Arnaud Bourdin; Cécile Chenivesse; Annick Clément; Emmanuel Gomez; Anne Gondouin; Sandrine Hirschi; François Lebargy; Charles-Hugo Marquette; David Montani; Grégoire Prévot; Sébastien Quetant; Martine Reynaud-Gaubert; Mathieu Salaun; Olivier Sanchez; Bruno Trumbic; Karim Berkani; Pierre-Yves Brillet; Marion Campana; Lara Chalabreysse; Gérard Chatté; Didier Debieuvre; Gilbert Ferretti; Jean-Michel Fourrier; Nicolas Just; Marianne Kambouchner; Bertrand Legrand; Frédéric Le Guillou; Jean-Pierre Lhuillier; Anas Mehdaoui; Jean-Marc Naccache; Catherine Paganon; Martine Rémy-Jardin; Salim Si-Mohamed; Philippe Terrioux; OrphaLung network

doi:10.1016/j.resmer.2022.100948

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

Respir Med Res. 2023 Jun:83:100948. doi: 10.1016/j.resmer.2022.100948. Epub 2022 Aug 4.

Authors

Vincent Cottin¹, Philippe Bonniaud², Jacques Cadranel³, Bruno Crestani⁴, Stéphane Jouneau⁵, Sylvain Marchand-Adam⁶, Hilario Nunes⁷, Lidwine Wémeau-Stervinou⁸, Emmanuel Bergot⁹, Elodie Blanchard¹⁰, Raphaël Borie⁴, Arnaud Bourdin¹¹, Cécile Chenivesse¹², Annick Clément¹³, Emmanuel Gomez¹⁴, Anne Gondouin¹⁵, Sandrine Hirschi¹⁶, François Lebargy¹⁷, Charles-Hugo Marquette¹⁸, David Montani¹⁹, Grégoire Prévot²⁰, Sébastien Quetant²¹, Martine Reynaud-Gaubert²², Mathieu Salaun²³, Olivier Sanchez²⁴, Bruno Trumbic²⁵, Karim Berkani²⁶, Pierre-Yves Brillet²⁷, Marion Campana²⁸, Lara Chalabreysse²⁹, Gérard Chatté³⁰, Didier Debieuvre³¹, Gilbert Ferretti³², Jean-Michel Fourrier³³, Nicolas Just³⁴, Marianne Kambouchner³⁵, Bertrand Legrand³⁶, Frédéric Le Guillou³⁷, Jean-Pierre Lhuillier³⁸, Anas Mehdaoui³⁹, Jean-Marc Naccache⁴⁰, Catherine Paganon⁴¹, Martine Rémy-Jardin⁴², Salim Si-Mohamed⁴³, Philippe Terrioux⁴⁴; OrphaLung network

Affiliations

¹ 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. Electronic address: vincent.cottin@chu-lyon.fr.
² 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.
³ Constitutive Reference Center for Rare Lung Diseases, Assistance Publique-Hôpitaux de Paris (AP-HP), Tenon Hospital, Sorbonne University, GRC 04 Theranoscan, Paris, France.
⁴ Constitutive Reference Center for Rare Lung Diseases, Pneumonology Department A, AP-HP, Bichat Hospital, Paris, France.
⁵ Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, Pontchaillou Hospital, IRSET UMR1085, Rennes 1 University, Rennes, France.
⁶ Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, CHRU, Bretonneau Hospital, Tours, France.
⁷ Constitutive Reference Center for Rare Lung Diseases, Pneumonology Department, Avicenne Hospital, Bobigny, AP-HP, Sorbonne Paris Nord University, Bobigny, France.
⁸ Constitutive Reference Center for Rare Lung Diseases, Heart-Lung Institute, Pneumonology and Immuno-allergology Department, CHRU of Lille, Lille, France.
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, France.
¹⁰ Expert Center for Rare Lung Diseases in Adults, Pneumonology Department, Haut Levêque Hospital, Bordeaux University Hospital Center, Pessac, France.
¹¹ 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.
¹² 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.
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, Sorbonne University, Paris, France.
¹⁴ Expert Center for Rare Lung Diseases, Pneumonology Department, Brabois Hospitals, Nancy Regional University Hospital Center, Vandoeuvre-les Nancy, France.
¹⁵ Expert Center for Rare Lung Diseases, Pneumonology Department, HP, Jean Minjoz Univerty Hospital Center, Besançon, France.
¹⁶ Expert Center for Rare Lung Diseases, Pneumonology Department, New Civil Hospital, Strasbourg, France.
¹⁷ Expert Center for Rare Lung Diseases, Pneumonology Department, Maison Blanche University Hospital Center, Reims, France.
¹⁸ 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.
¹⁹ 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.
²⁰ Expert Center for Rare Lung Diseases, Pneumonology Department, Larrey University Hospital Center, Toulouse, France.
²¹ Expert Center for Rare Lung Diseases, Pneumonology and Physiology Department, Grenoble Alpes University Hospital Center, Grenoble, France.
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, France.
²³ 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.
²⁴ Expert Center for Rare Lung Diseases, Pneumonology and Intensive Care Department, Georges Pompidou European Hospital, AP-HP, Paris, France.
²⁵ Cap Evidence, Paris, France.
²⁶ Pierre de Soleil Clinic, Vetraz Monthoux, France.
²⁷ UPRES EA 2363, Radiology Department, AP-HP, Avicenne Hospital, Paris 13 University, Bobigny, France.
²⁸ Pneumonology and Thoracic Oncology Department, Orléans Regional Hospital Center, Orléans, France.
²⁹ Pathological Anatomy Department, East Hospital Group, HCL, Bron, France.
³⁰ Pneumonology Practice and Protestant Infirmary, Caluire, France.
³¹ Pneumonology Department, GHRMSA, Emile Muller Hospital, Mulhouse, France.
³² Diagnostic and Interventional Radiology Department, Grenoble Alpes University Hospital Center, Grenoble Alpes University, Grenoble, France.
³³ Pierre Enjalran Association for Idiopathic Pulmonary Fibrosis (APEFPI), Meyzieu, France.
³⁴ Pneumonology Department, Victor Provo Hospital Center, Roubaix, France.
³⁵ Pathology Department, AP-HP, Avicenne Hospital, Bobigny, France.
³⁶ Medical Practice of Bourgogne, ULR 2694 METRICS, CERIM, Lille University, Lille University Hospital Center, Tourcoing, Lille, France.
³⁷ Pneumonology Practice, Esquirol Health Pole, Le Pradet, France.
³⁸ Pneumonology Practice, La Varenne Saint-Hilaire, France.
³⁹ Pneumonology and Thoracic Oncology Department, Eure-Seine Hospital Center, Évreux, France.
⁴⁰ Pneumonology, Allergology and Thoracic Oncology Department, Paris Saint-Joseph Hospital Group, Paris, France.
⁴¹ Pneumonology Department, Louis Pradel Hospital, HCL, Bron, France.
⁴² Heart-Lung Institute, Radiology and Thoracic Imaging Department, Lille Regional University Hospital Center, Lille, France.
⁴³ 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.
⁴⁴ Pneumonology Practice, Meaux, France.

PMID: 36630775
DOI: 10.1016/j.resmer.2022.100948

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.

Keywords: Biopsy; Interstitial lung disease; Pulmonary fibrosis; Usual interstitial pneumonia.

Publication types

Review
Practice Guideline

MeSH terms

Humans
Idiopathic Pulmonary Fibrosis* / diagnosis
Idiopathic Pulmonary Fibrosis* / epidemiology
Idiopathic Pulmonary Fibrosis* / therapy
Lung / pathology
Lung Transplantation*
Prognosis
Tomography, X-Ray Computed / methods