Idiopatisk lungfibros: klinik, mekanismer och prediktiva faktorer

Bakgrund:

Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease with progressive connective tissue transformation in the lung leading to shortness of breath, cough and impaired quality of life. The diagnosis is based on a typical HRCT (high resolution computed tomography) pattern and/or tissue specimen from the lung. The prognosis is poor with an expected median survival of 3-5-years.

The aetiology is unknown and there are no markers available predicting mortality or outcomes. There are currently two drugs avaliable on the market which slow down disease progression, but real-world data on the effect and quality-of-life are sparse. Målsättning:

The objective of the Ph.D.-project is to study the course of the disease and to identify factors that can predict outcome. Potential biomarkers and autoantibodies will be analyzed in biobank samples. In addition, co-morbidities and quality of life will be investigated as will effects of antifibrotic medications.

Arbetsplan:

Data from the Swedish IPF registry, with a linked biobank, will be used, including more than 650 patients. Lung function, co-morbidities, quality of life, treatments, side effects and disease course will be extracted from the register. Potential biomarkers and autoantibodies will be analyzed in a collaborative project with Lund University (Lung biology) and Rheumatology department at Karolinska Institutet.

Betydelse:

Idiopathic pulmonary fibrosis is a serious disease where early diagnosis and follow-up is cruical. There are currently no treatments that can restore lung function. Disease course, quality of life and prognostic factors are incompletely known.

By using real-world data from the Swedish IPF-registry we aim to gain important knowledge on early diagnosis, disease course, quality of life and potential biomarkers. Also, the effect of medications on these parameters will be assessed. The project can lead to improved diagnostics and follow-up.