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01.12.2015 | Research article | Ausgabe 1/2015 Open Access

BMC Pulmonary Medicine 1/2015

Re-evaluation of diagnostic parameters is crucial for obtaining accurate data on idiopathic pulmonary fibrosis

BMC Pulmonary Medicine > Ausgabe 1/2015
Jaana Kaunisto, K. Kelloniemi, E. Sutinen, U. Hodgson, A. Piilonen, R. Kaarteenaho, R. Mäkitaro, M. Purokivi, E. Lappi-Blanco, S. Saarelainen, H. Kankaanranta, A. Mursu, M. Kanervisto, E-R. Salomaa, M. Myllärniemi
Wichtige Hinweise

Competing interests

JK reports personal fees and non-financial support from Intermune/Roche, outside the submitted work. E-RS reports personal fees from Intermune, outside the submitted work. SS reports non-financial support from Intermune/Roche, outside the submitted work. HK reports grants from Tampere Tuberculosis Foundation, The Competitive State Research Financing of the Expert Responsibility Area of Tampere University Hospital, during the conduct of the study; personal fees and non-financial support from Almirall, personal fees from AstraZeneca, Chiesi Pharma AB, personal fees from GlaxoSmithKline, Leiras-Takeda, MSD, Novartis, Mundipharma, Medith, Resmed Finland, Roche, personal fees and non-financial support from Boehringer-Ingelmheim, non-financial support from Intermune, outside the submitted work. RM reports personal fees from lecture fee by Leiras-Takeda, non-financial support from British Thoracic Society winter meeting 2014 by GlaxoSmithKline, non-financial support from ERS 2013 Barcelona by Mundipharma, personal fees from lecture fee by Lilly, personal fees from advisory board member Boehringer-Ingelheim, outside the submitted work. RK reports non-financial support from Boehringer-Ingelheim and Intermune, outside the submitted work. AM reports personal fees from Intermune, personal fees from Leiras, outside the submitted work; MM reports grants, personal fees and other from Intermune/Roche, during the conduct of the study. EL-B reports grants from the Finnish Anti-Tuberculosis Association Foundation, non-financial support and personal fee for advisory board member from Pfizer, lecture fee from Eli Lilly Finland, all outside the submitted work.

Authors’ contributions

MM established the FinnishIPF –registry project and was the originator of the study. All authors have been involved with the patient recruitment. MM, ES, RK, E-RS, UH, JK, MK and AM participated to the clinical re-evaluation. KK and AP re-evaluated all the diagnostic HRCTs. RK and EL-B re-evaluated the surgical lung biopsies. MM, MK and ES checked and cleaned the data. MK made the statistical analyses while JK worked as an assistant. JK, KK, MK and MM produced the first draft of the paper. All authors revised the manuscript critically for important intellectual content and contributed to the final draft. All authors wrote the final approval of the version to be published. All authors agreed for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.



The FinnishIPF registry is a prospective, longitudinal national registry study on the epidemiology of idiopathic pulmonary fibrosis (IPF). It was designed to describe the characteristics, management and prognosis of prevalent and incident IPF patients. The study was initiated in 2012.


We present here results limited to five university hospitals. Patients with IPF were screened from hospital registries using ICD-10 diagnosis codes J84.1 and J84.9. All patients who gave informed consent were included and evaluated using novel diagnostic criteria. Point prevalence on the 31st of December in 2012 was calculated using the reported population in each university hospital city as the denominator.


Patients with ICD-10 codes J84.1 and J84.9 yielded a heterogeneous group – on the basis of patient records assessed by pulmonologists only 20–30 % of the cases were IPF. After clinical, radiological and histological re-evaluation 111 of 123 (90 %) of patients fulfilled the clinical criteria of IPF. The estimated prevalence of IPF was 8.6 cases/100 000. 60.4 % were men. Forty four percent of the patients were never-smokers. At diagnosis, the patients’ mean age was 73.5 years and mean FVC was 80.4 % and DLCO 57.3 % of predicted.


Our results suggest that hospital registries are inaccurate for epidemiological studies unless patients are carefully re-evaluated. IPF is diagnosed in Finland at a stage when lung function is still quite well preserved. Smoking in patients with IPF was less common than in previous reports.
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