Abstract
Background: Management of rare diseases including idiopathic pulmonary fibrosis (IPF) has been organised in France by the National plan for rare diseases. Clinical care in the community is coordinated by one national reference centre and nine regional competence centres.
Objective: To evaluate current diagnostic and treatment practice for IPF in the context of recent international guidelines.
Methods: A questionnaire (26 questions) has been submitted over the phone and on-line between December 7, 2011 and February 18, 2012, to 1,456 pulmonologists (out of 2,682 in France).
Results: 509 pulmonologists (35%) 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 centres, 141 (35%) indicated referring patients to those centres. The 2011 ATS/ERS/JRS/ALAT guidelines were known by 67% of pulmonologists involved in IPF, 84% of whom considered them appropriate for practice. An estimate of 58% of patients were diagnosed with mild to moderate IPF as defined by % predicted FVC ≥50% and % predicted DLCO ≥35%.
Conclusion: Despite correct awareness of international IPF guidelines, modalities of multidisciplinary discussion and of early diagnosis need to be improved through the network of expert centres.
Funding: Intermune France.
- © 2012 ERS