A consortium led by Prof. Dr. Michael Kreuter, Heidelberg, and involving Prof. Dr. Barbro Melgert, , Groningen, Prof. Dr. Janette K. Burgess, Groningen, Dr. Nicolas Kahn, Heidelberg, Prof. Dr. Marlies Wijsenbeek, Rotterdam, Dr. Mirjam Kool, Rotterdam as co-PIs obtained funding from the German Lung Fibrosis Support Group.
Interstitial lung diseases (ILDs) encompass a heterogeneous group of disorders characterised by varying degrees of inflammation and fibrosis. Idiopathic pulmonary fibrosis (IPF) is the most known progressive, fibrosing ILD. However, a large group of other ILDs often have a progressive fibrosing phenotype such as connective tissue disease (CTD)-associated ILD (including rheumatoid arthritis (RA) and systemic sclerosis (SSC)), chronic hypersensitivity pneumonitis (HP) and others. Like IPF, these diseases have high and progressive symptom burdens that impact on patient’s lives. The availability of anti-fibrotic drugs, and the advances in new immunomodulatory and biological therapies, has added to the complexity of therapeutic decision-making in progressive fibrotic lung diseases. Profiling of inflammatory and fibrotic blood markers will enable diagnosis of disease-underlying mechanisms and lead to tailored care for symptoms relief and optimized outcomes.
The balance between fibrotic and inflammatory components differs between pulmonary fibrotic disease endotypes and this equilibrium may change during disease phases, potentially contributing to disease progression. This project will examine a panel of potential biomarkers, in blood, and compare these to physiological parameters, including lung function and HRCT, and to patient reported outcomes on health-related quality of life, and symptoms.
Through identifying which biomarkers, or combination thereof, identifies patients at risk of (rapidly) progressing to fibrosis, which are the best indicators for disease worsening, which are related to specific symptoms, and which are influenced by current treatment options, we will advance potential for individualised treatments with maximum relief of symptoms and minimum harm.