Progressive Pulmonary Fibrosis in Connective Tissue Disease

Chiu, Yu-Hsiang

Prof.dr. J.M. (Jaap) van Laar
Dr. J. (Julia) Spierings & dr. M. (Mareye) Voortman
Research group:
July 2, 2024
10:15 h


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This thesis focuses on understanding and managing connective tissue diseases associated interstitial lung disease (CTD-ILD). The aim is to identify risk factors and biomarkers for progressive pulmonary fibrosis (PPF) and mortality in CTD-ILD patients. The thesis identified that older age, smoking, extensive fibrosis, and poor lung function were associated with higher mortality. The three criteria for defining PPF showed similar prognostication, with better performance in certain patient groups. Serum biomarkers were explored, decrease in CXCL11, CTGF and KL-6 were associated with improved pulmonary outcomes. The thesis reviewed the potential benefit and risk of hematopoietic stem cell transplantation in rapid progression of systemic sclerosis-associated ILD. Profibrotic mechanisms in systemic sclerosis, including cellular senescence and endothelial-to-mesenchymal transition, were evaluated. The thesis provides valuable insights for risk assessment, biomarker identification, and potential treatment strategies for CTD-ILD patients.