Email updates

Keep up to date with the latest news and content from BMC Pulmonary Medicine and BioMed Central.

Open Access Highly Accessed Research article

Idiopathic pulmonary fibrosis - a systematic review on methodology for the collection of epidemiological data

Jaana Kaunisto12, Eija-Riitta Salomaa12, Ulla Hodgson3, Riitta Kaarteenaho456 and Marjukka Myllärniemi37*

Author Affiliations

1 Division of Medicine, Pulmonary Diseases, Turku University Hospital, Turku, Finland

2 Department of Pulmonary Diseases and Clinical Allergology, University of Turku, Turku, Finland

3 Division of Pulmonary Medicine, Heart and Lung Center, Helsinki University Central Hospital, Helsinki, Finland

4 Center for Medicine and Clinical Research, Division of Respiratory Medicine, Kuopio University Hospital, Kuopio, Finland

5 Unit of Medicine and Clinical Research, Pulmonary Division, University of Eastern Finland, Kuopio, Finland

6 Respiratory Research Unit and Medical Research Center Oulu, Oulu University Hospital, Oulu, Finland

7 Departement of Clinical Medicine, Division of Pulmonary Medicine, University of Helsinki, Biomedicum, PoBox 63, 00014, Helsinki C405b, Finland

For all author emails, please log on.

BMC Pulmonary Medicine 2013, 13:53  doi:10.1186/1471-2466-13-53

Published: 20 August 2013



Recent studies suggest that the incidence of idiopathic pulmonary fibrosis (IPF) is rising. Accurate epidemiological data on IPF, however, are sparse and the results of previous studies are contradictory. This study was undertaken to gain insight into the various methods used in the epidemiological research of IPF, and to get accurate and comparable data on these different methodologies.


A systematic database search was performed in order to identify all epidemiological studies on IPF after the previous guidelines for diagnosis and treatment were published in 2000. Medline (via Pubmed), Science Sitation Index (via Web of Science) and Embase databases were searched for original epidemiological articles published in English in international peer-reviewed journals starting from 2001. After pre-screening and a full-text review, 13 articles were accepted for data abstraction.


Three different methodologies of epidemiological studies were most commonly used, namely: 1) national registry databases, 2) questionnaire-based studies, and 3) analysis of the health care system’s own registry databases. The overall prevalence and incidence of IPF varied in these studies between 0.5–27.9/100,000 and 0.22–8.8/100,000, respectively. According to four studies the mortality and incidence of IPF are rising.


We conclude that there are numerous ways to execute epidemiological research in the field of IPF. This review offers the possibility to compare the different methodologies that have been used, and this information could form a basis for future studies investigating the prevalence and incidence of IPF.

Interstitial lung disease; Incidence; Prevalence; Mortality; Register