RT Journal Article
SR Electronic
T1 Preliminary results of the French national prospective cohort on IPF
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP p646
VO 38
IS Suppl 55
A1 Hilario Nunes
A1 Zohra Carton
A1 Vincent Cottin
A1 Dominique Israel-Biet
A1 Michel Brauner
A1 Marianne Kambouchner
A1 Bruno Crestani
A1 Jacques Cadranel
A1 Bruno Wallaert
A1 Grégoire Prévot
A1 Karine Juvin
A1 Raphael Borie
A1 Marie Wislez
A1 Juliette Chabrol
A1 Anne Gaudouin
A1 Severine Feuillet
A1 Frederic Gagnadoux
A1 Sylvain Marchand-Adam
A1 Bernard Maître
A1 Philippe Bonniaud
A1 Philippe Delaval
A1 Jean-François Cordier
A1 Dominique Valeyre
YR 2011
UL http://erj.ersjournals.com/content/38/Suppl_55/p646.abstract
AB Introduction: Owing the absence of published prospective cohorts, the epidemiology and natural history of IPF remains unclear.Aims: To determine the factors associated with the occurrence of pre-defined evolutive events: slow progression of IPF, acute exacerbation (AE), subacute exacerbation (subAE), pulmonary hypertension (PH) and death.Methods: Prospective cohort involving all the 24 French University Hospitals. The diagnosis of IPF was based on 2000 ATS/ERS criteria and centrally reviewed.Results: From 12/2007 to 12/2010, 240 incident cases of IPF (diagnosis <9 months) were included. Data are available for 210 patients (men: 80%; age: 69±10 years; non-smokers: 29%). At inclusion, a history of neoplasia was reported in 13% and at least one cardiovascular disease in 29% (coronaropathy: 18%). BMI was 27.5±4.3 and it was >25 in 68%. A familial form of IPF was noted in 8%. A combined pulmonary fibrosis and emphysema syndrome was seen in 18% and asymetrical IPF in 7%. Baseline FVC was 76±21% and DLCO was 47±17%. On BAL, lymphocytes were ≥30% in 4.1% and eosinophils ≥20% in 2.1%. Surgical lung biopsy was performed in 31%. After a follow-up of 14±9 months (range: 1-36 months) following events were observed: 34 slow progressions, 19 AE, 10 subAE, 9 PH and 46 deaths. Eight patients were transplanted. The incidence of AE was 12% at 1 year. Survival was 80% and 63% at 1 and 2 years. Mortality was related to respiratory causes in 78% (AE: 37% and end-stage respiratory insufficiency: 26%).Conclusion: Our results confirm the high frequency of overweight and severe co-morbidities in IPF. Despite a relatively low incidence of evolutive events, mortality is already high, with AE being the major cause of death.