%0 Journal Article %T French recommendations for the diagnosis and management of lymphangioleiomyomatosis %+ Infections Virales et Pathologie Comparée - UMR 754 (IVPC) %+ Centre de recherche Cardio-Thoracique de Bordeaux [Bordeaux] (CRCTB) %+ CHU Pontchaillou [Rennes] %+ Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV) %+ Assistance Publique - Hôpitaux de Marseille (APHM) %+ Microbes évolution phylogénie et infections (MEPHI) %+ AP-HP - Hôpital Bichat - Claude Bernard [Paris] %+ Physiopathologie et Epidémiologie des Maladies Respiratoires (PHERE (UMR_S_1152 / U1152)) %+ Plateforme F-CRIN %+ Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P - U1272 Inserm) %+ Hôpital Avicenne [AP-HP] %+ Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille) %+ CHU Amiens-Picardie %+ Agents infectieux, résistance et chimiothérapie - UR UPJV 4294 (AGIR ) %+ EA3920 University of Burgundy Franche-Comté Besançon %+ Equipe CADIR (LNC - U1231) %+ Laboratoire de Biologie Tissulaire et d'ingénierie Thérapeutique UMR 5305 (LBTI) %+ Hôpital Louis Pradel [CHU - HCL] %+ Centre pour l'innovation en cancérologie de Lyon (CICLY) %+ Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E) %+ Institute for Advanced Biosciences / Institut pour l'Avancée des Biosciences (Grenoble) (IAB) %+ Centre Hospitalier Universitaire [Grenoble] (CHU) %A Cottin, Vincent %A Blanchard, Elodie %A Kerjouan, Mallorie %A Lazor, Romain %A Reynaud-Gaubert, Martine %A Taille, Camille %A Uzunhan, Yurdagül %A Wemeau, Lidwine %A Andrejak, Claire %A Baud, Dany %A Bonniaud, Philippe %A Brillet, Pierre-Yves %A Calender, Alain %A Chalabreysse, Lara %A Court-Fortune, Isabelle %A Desbaillets, Nicolas Pierre %A Ferretti, Gilbert %A Guillemot, Anne %A Hardelin, Laurane %A Kambouchner, Marianne %A Leclerc, Violette %A Lederlin, Mathieu %A Malinge, Marie-Claire %A Mancel, Alain %A Marchand-Adam, Sylvain %A Maury, Jean-Michel %A Naccache, Jean-Marc %A Nasser, Mouhamad %A Nunes, Hilario %A Pagnoux, Gaële %A Prévot, Grégoire %A Rousset-Jablonski, Christine %A Rouviere, Olivier %A Si-Mohamed, Salim %A Touraine, Renaud %A Traclet, Julie %A Turquier, Ségolène %A Vagnarelli, Stéphane %A Ahmad, Kaïs %< avec comité de lecture %@ 2590-0412 %J Respiratory Medicine and Research %I Elsevier %V 83 %P 101010 %8 2023-06 %D 2023 %R 10.1016/j.resmer.2023.101010 %M 37087906 %Z Life Sciences [q-bio]/Human health and pathologyJournal articles %X BackgroundThe present article is an English-language version of the French National Diagnostic and Care Protocol, a pragmatic tool to optimize and harmonize the diagnosis, care pathway, management and follow-up of lymphangioleiomyomatosis in France.MethodsPractical recommendations were developed in accordance with the method for developing a National Diagnosis and Care Protocol for rare diseases of the Haute Autorité de Santé and following international guidelines and literature on lymphangioleiomyomatosis. It was developed by a multidisciplinary group, with the help of patient representatives and of RespiFIL, the rare disease network on respiratory diseases.ResultsLymphangioleiomyomatosis is a rare lung disease characterised by a proliferation of smooth muscle cells that leads to the formation of multiple lung cysts. It occurs sporadically or as part of a genetic disease called tuberous sclerosis complex (TSC). The document addresses multiple aspects of the disease, to guide the clinicians regarding when to suspect a diagnosis of lymphangioleiomyomatosis, what to do in case of recurrent pneumothorax or angiomyolipomas, what investigations are needed to make the diagnosis of lymphangioleiomyomatosis, what the diagnostic criteria are for lymphangioleiomyomatosis, what the principles of management are, and how follow-up can be organised. Recommendations are made regarding the use of pharmaceutical specialties and treatment other than medications.ConclusionThese recommendations are intended to guide the diagnosis and practical management of pulmonary lymphangioleiomyomatosis. %G English %L hal-04083634 %U https://u-picardie.hal.science/hal-04083634 %~ UNIV-PARIS13 %~ UNIV-BOURGOGNE %~ HCL %~ EPHE %~ UNIV-TLSE3 %~ UGA %~ CNRS %~ UNIV-AMU %~ UNIV-PICARDIE %~ UNIV-LYON1 %~ APHP %~ HL %~ PSL %~ LNC-UMR866 %~ TEST-DEV %~ IVPC %~ UDL %~ UNIV-LYON %~ INRAE %~ SORBONNE-PARIS-NORD %~ UNIV-PARIS %~ UNIVERSITE-PARIS %~ UP-SANTE %~ EPHE-PSL %~ UGA-EPE %~ U-PICARDIE %~ AGIR-UPJV %~ UNIV-UT3 %~ UT3-INP %~ UT3-TOULOUSEINP %~ RESEAU-EAU %~ INSTITUT-AGRO