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.
Keywords: Drug therapy; Idiopathic pulmonary fibrosis; Management; Pirfenidone; Survey.
Copyright © 2013. Published by Elsevier Ltd.