Idiopathic pulmonary fibrosis in Sweden: report from the first year of activity of the Swedish IPF-Registry.
BACKGROUND
Idiopathic pulmonary fibrosis (IPF) is an emerging problem in the western world, being related to increasing age and implying significant costs for the diagnosis and management of affected patients. The epidemiology of IPF is not well understood.


METHODS
To allow estimates of the problem and eventually to evaluate quality of the care of IPF patients in Sweden, a national IPF Registry was started in the autumn of 2014. Data on criteria used to diagnose IPF, demographics, lung function, and quality of life (measured with the King's Brief Interstitial Lung Disease Questionnaire, K-BILD) were reported directly to the registry, based at the coordinating centre (Karolinska University Hospital, Stockholm, Sweden) via a web-based platform.


RESULTS
During the first year, the registry was implemented in 11 (33%) of the 33 respiratory units in the country. Seventy-one patients were registered between October 2014 and October 2015, 50 (70.4%) males and 21 (29.6%) females. Median age was 70 (range 47-86). The mean K-BILD score at the first inclusion in the registry was 54.3+9.5.


CONCLUSIONS
The main features of IPF patients in this first Swedish cohort were consistent with data published in the literature in main multinational randomized controlled trials. The K-BILD questionnaire showed that quality of life of patients with IPF and their perception of the disease are quite poor at the time of inclusion in the registry.
Department of Respiratory Medicine and Allergy, Karolinska University Hospital, Stockholm, Sweden.|Respiratory Medicine Unit, Department of Medicine, Solna and Center for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden; giovanni.ferrara@ki.se.
Giovanni
G
Ferrara
Department of Respiratory Medicine and Allergy, Karolinska University Hospital, Stockholm, Sweden.
Lisa
L
Carlson
Gävle Hospital, Gävle, Sweden.
Andreas
A
Palm
Skåne University Hospital, Malmö, Sweden.|Department of Respiratory Medicine and Allergology, Skåne University Hospital, Lund University, Lund, Sweden.
Jonas
J
Einarsson
Sunderby Hospital, Luleå, Sweden.
Cecilia
C
Olivesten
Department of Respiratory Medicine and Allergy, Karolinska University Hospital, Stockholm, Sweden.|Respiratory Medicine Unit, Department of Medicine, Solna and Center for Molecular Medicine, Karolinska Institutet, Stockholm, Sweden.
Magnus
M
Sköld
K-BILD; idiopathic pulmonary fibrosis; lung function; quality of life; registry