Course of disease and cause of death in patients with idiopathic pulmonary fibrosis in Eastern Finland

Abstract

Background: Idiopathic pulmonary fibrosis (IPF) reveal unpredictable course of disease although mortality rate is usually high. Aims: We aimed to evaluate the course of disease, outcome and causes of death among patients with IPF. Methods: Demographic information was collected from 223 patients with pulmonary fibrosis treated in the Kuopio University Hospital between 2002-2012. Radiological, clinical and histological material was re-analyzed by pulmonologists, radiologists and pathologists. Data was collected on specially designed forms. After re-evaluation, 131 patients with IPF were included into the study. The patients were categorized into three groups according to the course of the disease, i.e. slow, moderate and fast progression. Prognosis was estimated using the GAP-index. Concomitant diseases and causes of death were gathered. Results: The study population comprised of 95 men and 36 women of which 35 % were nonsmokers. Median survival of the patients was 35 months. The course of the disease was rapid in 30%, moderate in 28% and slow in 29% of the patients. The GAP model predicted well 1- and 2-year mortality risk in GAP-stages I and II, but underestimated it in stage III. The most common concomitant diseases were coronary artery disease (CAD) (47%), hypertension (45%) and diabetes (26%). The most common causes of death were IPF (57%), CAD (11%) and lung cancer (5%). Conclusion: Even though the prognosis of IPF is overall poor, 29% of the patients revealed a slowly progressive disease characterized by unpredictable survival. Although CAD is very common in Finnish IPF patients, the most common cause of death was IPF.