V Cottin1, J Cadranel2, B Crestani3, J C Dalphin4, P Delaval5, D Israel-Biet6, R Kessler7, M Reynaud-Gaubert8, D Valeyre9, B Wallaert10, B Bouquillon11, J F Cordier12. 1. National Reference Centre for Rare Pulmonary Diseases, Louis Pradel University Hospital, Lyon, France. Electronic address: vincent.cottin@chu-lyon.fr. 2. Competence Centre for Rare Pulmonary Diseases, AP-HP, Tenon Hospital, Pierre & Marie Curie University, Paris, France. 3. Competence Centre for Rare Pulmonary Diseases, Bichat Hospital, Paris, France. 4. Competence Centre for Rare Pulmonary Diseases, University Hospital, Besançon, France. 5. Competence Centre for Rare Pulmonary Diseases, University Hospital, Rennes, France. 6. Competence Centre for Rare Pulmonary Diseases, AP-HP, Georges Pompidou European Hospital, Paris Descartes University, Paris, France. 7. Competence Centre for Rare Pulmonary Diseases, University Hospital, Strasbourg, France. 8. Competence Centre for Rare Pulmonary Diseases, University Hospital, Marseille, France. 9. Competence Centre for Rare Pulmonary Diseases, AP-HP, Avicenne Hospital, and Université Paris 13, Sorbonne Paris Cité, Bobigny, France. 10. Competence Centre for Rare Pulmonary Diseases, University Hospital, Lille, France. 11. Opened Mind Health, Roubaix, France. 12. National Reference Centre for Rare Pulmonary Diseases, Louis Pradel University Hospital, Lyon, France.
Abstract
BACKGROUND: The present survey coordinated by the French expert centres for rare pulmonary diseases investigated French pulmonologists' current diagnostic and therapeutic practice for idiopathic pulmonary fibrosis (IPF). METHODS: From December 7, 2011 to February 18, 2012, all French pulmonologists (n = 2608) were contacted. Those who reported following up at least one IPF patient (n = 509) were administered a 26-item questionnaire by phone or e-mail. RESULTS: 509 pulmonologists (41% of responders, 20% of French pulmonologists) were involved in the management of IPF patients. Of those, 36% discussed the cases with radiologists and pathologists. Out of 406 community pulmonologists practicing outside of reference or competence (e.g. expert) centres, 141 (35%) indicated referring patients to those centres. The 2011 international guidelines for IPF were known by 67% of pulmonologists involved in IPF, 84% of whom considered them appropriate for practice. About 58% of patients were diagnosed with mild to moderate IPF as defined by percentage predicted forced vital capacity ≥50% and percentage predicted diffusing capacity of the lung for carbon monoxide ≥35%. Management resulted from multidisciplinary discussion in 36% of the cases. By the end of December 2011, 49% of patients with mild to moderately severe IPF were treated with oral corticosteroids, and 27% received no treatment. CONCLUSIONS: Despite correct awareness of international IPF guidelines, modalities of multidisciplinary discussion and of early diagnosis and management need to be improved through the network of expert centres.
BACKGROUND: The present survey coordinated by the French expert centres for rare pulmonary diseases investigated French pulmonologists' current diagnostic and therapeutic practice for idiopathic pulmonary fibrosis (IPF). METHODS: From December 7, 2011 to February 18, 2012, all French pulmonologists (n = 2608) were contacted. Those who reported following up at least one IPF patient (n = 509) were administered a 26-item questionnaire by phone or e-mail. RESULTS: 509 pulmonologists (41% of responders, 20% of French pulmonologists) were involved in the management of IPF patients. Of those, 36% discussed the cases with radiologists and pathologists. Out of 406 community pulmonologists practicing outside of reference or competence (e.g. expert) centres, 141 (35%) indicated referring patients to those centres. The 2011 international guidelines for IPF were known by 67% of pulmonologists involved in IPF, 84% of whom considered them appropriate for practice. About 58% of patients were diagnosed with mild to moderate IPF as defined by percentage predicted forced vital capacity ≥50% and percentage predicted diffusing capacity of the lung for carbon monoxide ≥35%. Management resulted from multidisciplinary discussion in 36% of the cases. By the end of December 2011, 49% of patients with mild to moderately severe IPF were treated with oral corticosteroids, and 27% received no treatment. CONCLUSIONS: Despite correct awareness of international IPF guidelines, modalities of multidisciplinary discussion and of early diagnosis and management need to be improved through the network of expert centres.
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