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Prognosis and causes of death of patients with acute exacerbation of fibrosing interstitial lung diseases
  1. Johanna Salonen1,2,
  2. Minna Purokivi3,
  3. Risto Bloigu4 and
  4. Riitta Kaarteenaho1,2
  1. 1Respiratory Medicine, Research Unit of Internal Medicine, University of Oulu, Oulu, Finland
  2. 2Medical Research Center, Oulu University Hospital, Oulu, Finland
  3. 3The Center of Medicine and Clinical Research, Division of Respiratory Medicine, Kuopio University Hospital, Kuopio, Finland
  4. 4Medical Informatics and Statistics Research Group, University of Oulu, Oulu, Finland
  1. Correspondence to Dr Johanna Salonen; johanna.salonen{at}


Background The aim of this study was to compare the clinical characteristics, causes of death and factors impacting on the prognosis of patients with idiopathic pulmonary fibrosis (IPF) and other fibrosing interstitial lung disease (FILD) with a history of acute exacerbation (AE) of IPF or FILD.

Methods Retrospective data of hospital treatment periods caused by AE-IPF and AE-FILD were collected from medical records. Clinical features and survival data of IPF and non-IPF cases were evaluated and compared. The underlying and immediate causes of death were gathered from death certificates.

Results A total of 128 patients fulfilled the criteria for inclusion. IPF (n=79/62%), rheumatoid arthritis-associated interstitial lung disease (RA-ILD; n=17/14%) and asbestosis (n=11/8.6%) were the most common FILD subgroups in the study. The median survival after hospitalisation in AE-IPF was 2.6 months compared with 21 months in other AE-FILDs (p<0.001). The survival difference was not explained by age, gender or pulmonary function test results at the time of hospitalisation. Patients with non-specific interstitial pneumonia and RA-ILD had the most favourable prognosis. ILD was the most common underlying cause of death in both patients with IPF and with other FILD accounting for 87% and 78% of deaths, respectively.

Conclusions We detected a significantly longer survival in AE of patients with non-IPF compared with that of AE-IPFs. The prognosis of patients was affected by the underlying lung disease since pulmonary fibrosis was the underlying cause of death in the majority of all patients with FILD having experienced an AE.

  • idiopathic pulmonary fibrosis
  • interstitial lung disease
  • acute exacerbation
  • asbestosis
  • survival

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  • Contributors JS collected the study material, planned the data collection form, interpreted and analysed the data and prepared the draft of the manuscript. RB planned and participated in the statistical analyses. MP and RK participated in planning the data collection form, study design and the interpretation of the data. RK managed the study and contributed substantially to data interpretation by re-evaluating study patients and data from medical records. All authors participated in the preparation of the manuscript, read and approved the final manuscript.

  • Funding JS has received personal grants for scientific work from Foundation of the Finnish Anti-Tuberculosis Association and the Research Foundation of the Pulmonary Diseases HES. RK has received grants for the study group from Foundation of the Finnish Anti-Tuberculosis Association, the Research Foundation of the Pulmonary Diseases, Jalmari and Rauha Ahokas Foundation and the Research Foundation of North Finland.

  • Competing interests JS reports congress fees and travel costs from Boehringer-Ingelheim, Novartis, Orion Pharma, Ratiopharm and Roche, and lecture fees from Boehringer-Ingelheim, Chiesi, GlaxoSmithKline, Mundipharma, Orion Pharma and Roche outside the submitted work. MP reports lecture fee from Boehringer-Ingelheim Finland and congress fee and travel costs from Roche, outside the submitted work. RB has nothing to disclose. RK reports consultant fees from GlaxoSmithKline and Boehringer-Ingelheim, lecture fees from Roche and Boehringer-Ingelheim, and a congress travel costs from Orion outside the submitted work.

  • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

  • Patient consent for publication Not required.

  • Ethics approval The study protocol was approved by the Ethical Committee of the Northern Ostrobothnia Hospital District (statement 2/2015). Permission to use death certificates was given by Statistics Finland (Dnro: TK-53-515-15). The study was conducted in compliance with the Declaration of Helsinki.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data are available upon reasonable request. The datasets generated and analysed during the current study are not publicly available due to the relatively small population of Northern Finland since we could not guarantee individuals’ anonymity as the data were collected in a detailed manner, but it is available from the corresponding author on reasonable request.

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