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Adjudication committee
- First opponent: Professor Marlies Wijsenbeek, Erasmus MC – University Medical Center
- Second opponent: Professor Michael Kreuter, University of Heidelberg
- Third member and chair of the evaluation committee: Professor Helge Skulstad, University of Oslo
Chair of the Defence
Professor Tom Hemming Karlsen, Faculty of Medicine, University of Oslo
Summary
Idiopathic pulmonary fibrosis (IPF) and other fibrotic interstitial lung diseases (ILDs) are associated with substantial mortality, symptoms, and impairment in health-related quality of life (HRQL). Questions remain about clinical study endpoints, risk of diagnostic procedures, and outcomes associated with treatment.
This thesis addresses questions related to disease progression, mortality and HRQL outcomes in fibrotic ILDs. The studies were based on secondary analysis of prospective clinical trials, prospectively collected registry data, and a prospective cohort study.
The first study demonstrates the increased risk of mortality associated with acute hospital admission in IPF, independent of lung function decline, and shows that clinical endpoints such as hospital admissions may complement lung function endpoints in clinical trials.
The second study demonstrates that the risks of operative mortality and post-operative respiratory failure were generally moderate, but were higher among patients with poorer diffusion capacity (DLCO), those treated with corticosteroids and those with pulmonary hypertension.
In the third study, ILD-specific HRQL appeared to be worse in IPF than in systemic sclerosis-associated ILD, but similar after accounting for lower lung function in IPF, suggesting that lung function impairment is a primary driver of ILD-specific HQRL across multiple fibrotic ILDs.
The fourth study demonstrates similar decline in lung function, but substantially greater risk of mortality among patients with lower lung function at the time of IPF treatment initiation, as compared with patients with more moderately impaired lung function. This further highlights the importance of clinical events in addition to lung function endpoints in assessing the effect of ILD treatment.
These studies confirm the importance of a multifaceted approach to assessing disease status, progression and mortality risk in fibrotic ILDs.
Additional information
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