Demographic and clinical profile of idiopathic pulmonary fibrosis patients in Spain: the SEPAR National Registry

BackgroundLittle is known on the characteristics of patients diagnosed with idiopathic pulmonary fibrosis (IPF) in Spain. We aimed to characterize the demographic and clinical profile of IPF patients included in the IPF National Registry of the Spanish Respiratory Society (SEPAR).MethodsThis is a prospective, observational, multicentre and nationwide study that involved 608 IPF patients included in the SEPAR IPF Registry up to June 27th, 2017, and who received any treatment for their disease. IPF patients were predominantly males, ex-smokers, and aged in their 70s, similar to other registries.ResultsUpon inclusion, meanSD predicted forced vital capacity was 77.6%+/- 19.4, diffusing capacity for carbon monoxide was 48.5%+/- 17.7, and the 6-min walk distance was 423.5m +/- 110.4. The diagnosis was mainly established on results from the high-resolution computed tomography in the proper clinical context (55.0% of patients), while 21.2% of patients required invasive procedures (surgical lung biopsy) for definitive diagnosis. Anti-fibrotic treatment was prescribed in 69.4% of cases, 51.5% pirfenidone and 17.9% nintedanib, overall with a good safety profile.Conclusions The SEPAR IPF Registry should help to further characterize current characteristics and future trends of IPF patients in Spain and compare/pool them with other registries and cohorts

Cirrosis biliar primaria con neumonía organizada secundaria

Resume: Organized pneumonia, previously called bronchiolitis obliterans with organized pneumonia, is an interstitial lung disea- se that belongs to the group of idiopathic interstitial diseases and was first recognized in 1985. Sometimes it is not rela- ted to a specific cause calleing it in that case cryptogenetic, organized pneumonia but is frequently associated with other etiologies that may be infectious, inflammatory, neoplastic or secondary to drugs, it might also be related to extrapul- monary pathology as is the case of our patient in which is associated with a chronic cholestatic liver disease such as primary biliary cirrhosis