Fibrotic lung disease : early signs, treatment and outcomes

Abstract

Idiopathic pulmonary fibrosis (IPF) has gained a lot of attention by the research community in recent decades. Thanks to enhanced understanding on pathophysiological mechanisms behind the fibrosing process together with two approved treatments that decelerate the progress, the interest within the field has increased.

However, despite the success, IPF is still recognized as a devastating disease with inevitably progressive loss of lung function, disabling symptoms, impaired health-related quality of life (HRQoL) and a poor, unpredictable prognosis. While large controlled clinical trials have increased knowledge and enabled new treatments for IPF, they have failed to show a beneficial effect on HRQoL.

Many needs of the individual IPF patients are unmet. By gathering patient data in a registry, we can track patient characteristics, disease behavior and HRQoL longitudinally in a real-world healthcare setting with its strengths and limitations. By doing so, we can truly see how IPF is managed.

The aim of this thesis is to explore the disease pathway and management of IPF. The thesis is built around four studies. As an important quality index of IPF management, early diagnosis was highlighted and discussed by estimating a high prevalence of interstitial lung abnormalities, potential pre-stage for IPF, in a large population-based sample (study I). Study II and III gave an overview on current important gaps in IPF care. IPF was diagnosed at a moderate stage when its symptoms already impair HRQoL. Patients received antifibrotic treatment with a delay, and a significant proportion of patients were not treated at all. Most importantly, national differences were observed: only one third of Finnish IPF patients received treatment between 2014 and 2017, while two-thirds of Swedish IPF patients were treated during the same period. Additionally, study III demonstrated that antifibrotic treatment may stabilize HRQoL in IPF, especially symptoms related to progressive loss of lung function. In study IV, the prognostic value of six-minute walking test was confirmed.

In summary, many gaps in IPF care are identified and discussed, raising further questions for future studies.