European Respiratory Society guidelines for the Diagnosis and Management of Pulmonary Alveolar Proteinosis - Archive ouverte HAL
Article Dans Une Revue European Respiratory Journal Année : 2024

European Respiratory Society guidelines for the Diagnosis and Management of Pulmonary Alveolar Proteinosis

Cormac Mccarthy (1) , Francesco Bonella (2) , Marissa O'Callaghan (1) , Clairelyne Dupin (3, 4, 5) , Tiago Alfaro (6) , Markus Fally (7) , Raphaël Borie (3, 4, 5) , Ilaria Campo (8, 9) , Vincent Cottin (10, 11, 12) , Aurélie Fabre (13) , Matthias Griese (14) , Alice Hadchouel (4, 15, 16) , Stéphane Jouneau (17, 18) , Maria Kokosi (19, 20) , Effrosyni Manali (21) , Helmut Prosch (22) , Bruce Trapnell (23) , Marcel Veltkamp (24, 25) , Tisha Wang (25) , Ingrid Toews (26) , Alexander Mathioudakis (27, 28, 29) , Elisabeth Bendstrup (30)
1 UCD - University College Dublin [Dublin]
2 Ruhrlandklinik University Hospital
3 AP-HP - Hôpital Bichat - Claude Bernard [Paris]
4 AP-HP - Assistance publique - Hôpitaux de Paris (AP-HP)
5 PHERE (UMR_S_1152 / U1152) - Physiopathologie et Epidémiologie des Maladies Respiratoires
6 H.U.C. - Hospitais da Universidade de Coimbra
7 Copenhagen University Hospital
8 IRCCS - Istituti di Ricovero e Cura a Carattere Scientifico
9 Fondazione IRCCS Policlinico San Matteo [Pavia]
10 Hôpital Louis Pradel [CHU - HCL]
11 HCL - Hospices Civils de Lyon
12 IVPC - Infections Virales et Pathologie Comparée - UMR 754
13 St Vincent's University Hospital
14 LMU - Ludwig Maximilian University [Munich] = Ludwig Maximilians Universität München
15 UAR 3633 / US24 - Structure Fédérative de Recherche Necker
16 INEM - UM 111 (UMR 8253 / U1151) - Institut Necker Enfants-Malades
17 Irset - Institut de recherche en santé, environnement et travail
18 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
19 Guy's and St Thomas' NHS Foundation Trust
20 Royal Brompton Hospital
21 NKUA - National and Kapodistrian University of Athens
22 Medizinische Universität Wien = Medical University of Vienna
23 Cincinnati Children's Hospital Medical Center
24 St. Antonius Hospital [Nieuwegein]
25 UMCU - University Medical Center [Utrecht]
26 Albert-Ludwigs-Universität Freiburg = University of Freiburg
27 MAHSC - Manchester Academic Health Science Centre
28 MFT - Manchester University NHS Foundation Trust
29 University of Manchester [Manchester]
30 Aarhus University Hospital
Cormac Mccarthy
Connectez-vous pour contacter l'auteur
University College Dublin [Dublin]
Francesco Bonella
Ruhrlandklinik University Hospital
Marissa O'Callaghan
University College Dublin [Dublin]
Clairelyne Dupin
AP-HP - Hôpital Bichat - Claude Bernard [Paris]
Assistance publique - Hôpitaux de Paris (AP-HP)
Physiopathologie et Epidémiologie des Maladies Respiratoires
Tiago Alfaro
Hospitais da Universidade de Coimbra
Markus Fally
Copenhagen University Hospital
Raphaël Borie
AP-HP - Hôpital Bichat - Claude Bernard [Paris]
Assistance publique - Hôpitaux de Paris (AP-HP)
Physiopathologie et Epidémiologie des Maladies Respiratoires
Ilaria Campo
Istituti di Ricovero e Cura a Carattere Scientifico
Fondazione IRCCS Policlinico San Matteo [Pavia]
Vincent Cottin
Hôpital Louis Pradel [CHU - HCL]
Hospices Civils de Lyon
Infections Virales et Pathologie Comparée - UMR 754
Aurélie Fabre
St Vincent's University Hospital
Matthias Griese
Ludwig Maximilian University [Munich] = Ludwig Maximilians Universität München
Alice Hadchouel
Assistance publique - Hôpitaux de Paris (AP-HP)
Structure Fédérative de Recherche Necker
Institut Necker Enfants-Malades
Stéphane Jouneau
Institut de recherche en santé, environnement et travail
Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Pontchaillou]
Maria Kokosi
Guy's and St Thomas' NHS Foundation Trust
Royal Brompton Hospital
Effrosyni Manali
  • Fonction : Auteur
National and Kapodistrian University of Athens
Helmut Prosch
Medizinische Universität Wien = Medical University of Vienna
Bruce Trapnell
Cincinnati Children's Hospital Medical Center
Marcel Veltkamp
St. Antonius Hospital [Nieuwegein]
University Medical Center [Utrecht]
Tisha Wang
University Medical Center [Utrecht]
Ingrid Toews
Albert-Ludwigs-Universität Freiburg = University of Freiburg
Alexander Mathioudakis
Manchester Academic Health Science Centre
Manchester University NHS Foundation Trust
University of Manchester [Manchester]
Elisabeth Bendstrup
Aarhus University Hospital

Résumé

Background: Pulmonary alveolar proteinosis (PAP) is a rare syndrome caused by several distinct diseases leading to progressive dyspnoea, hypoxemia, risk of respiratory failure and early death due to accumulation of proteinaceous material in the lungs. Diagnostic strategies may include computed tomography (CT) of the lungs, bronchoalveolar lavage, evaluation of antibodies against granulocyte macrophage colony stimulating factor (GM-CSF), genetic testing, and, eventually, lung biopsy. The management options are focused at removing the proteinaceous material by whole lung lavage (WLL), augmentation therapy with GM-CSF, rituximab, plasmapheresis, and lung transplantation. The presented diagnostic and management guideline aim to provide guidance to physicians managing patients with PAP. Methods: A European Respiratory Society Task Force committee composed of clinicians, methodologists, and patients with experience in PAP developed recommendations in accordance with the ERS Handbook for Clinical Practice Guidelines and the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) approach. This included a systematic review of the literature and application of the GRADE approach to assess the certainty of the evidence and strength of recommendations. The committee formulated five PICO (Patients, Intervention, Comparison, Outcomes) questions, and two narrative questions to develop specific evidence-based recommendations. Results: The Task Force committee developed recommendations for five PICOs. These included management of PAP with WLL, GM-CSF augmentation therapy, rituximab, plasmapheresis, and lung transplantation. Also, the committee made recommendations regarding the use of GM-CSF antibody testing, diagnostic bronchoalveolar lavage (BAL) and biopsy based on narrative questions.In addition to the recommendations, the committee provided information on the hierarchy of diagnostic interventions and therapy. Conclusions: The diagnosis of PAP is based on CT and BAL cytology or lung histology, whereas diagnosis of specific PAP-causing diseases requires GM-CSF antibody testing or genetic analysis. There are several therapies including WLL and augmentation therapy with GM-CSF available to treat PAP, but supporting evidence is still limited.

Domaines

Pneumologie et système respiratoire

Laurent Jonchère  : Connectez-vous pour contacter le contributeur

https://hal.science/hal-04690353

Soumis le : vendredi 6 septembre 2024-13:53:04

Dernière modification le : jeudi 17 octobre 2024-10:52:03

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Dates et versions

hal-04690353 , version 1 (06-09-2024)

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Citer

Cormac Mccarthy, Francesco Bonella, Marissa O'Callaghan, Clairelyne Dupin, Tiago Alfaro, et al.. European Respiratory Society guidelines for the Diagnosis and Management of Pulmonary Alveolar Proteinosis. European Respiratory Journal, 2024, pp.2400725. ⟨10.1183/13993003.00725-2024⟩. ⟨hal-04690353⟩

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