Targeting interleukin-13 in idiopathic pulmonary fibrosis: from promising path to dead end

MS Wijsenbeek, M Kool, V Cottin - European Respiratory …, 2018 - Eur Respiratory Soc
MS Wijsenbeek, M Kool, V Cottin
European Respiratory Journal, 2018Eur Respiratory Soc
The pathogenesis of idiopathic pulmonary fibrosis (IPF) is characterised by repeated
subclinical injury to the alveolar epithelium, leading to injured alveoli, myofibroblast
recruitment and activation, resulting in aberrant wound healing with uncontrolled matrix
deposition and progressive fibrosis. However, inflammation and immunity are also thought
to play a modulatory role in IPF pathogenesis. Data from IPF patients and experimental
animal models have shown that type 2 inflammatory processes are activated in pulmonary …
The pathogenesis of idiopathic pulmonary fibrosis (IPF) is characterised by repeated subclinical injury to the alveolar epithelium, leading to injured alveoli, myofibroblast recruitment and activation, resulting in aberrant wound healing with uncontrolled matrix deposition and progressive fibrosis. However, inflammation and immunity are also thought to play a modulatory role in IPF pathogenesis. Data from IPF patients and experimental animal models have shown that type 2 inflammatory processes are activated in pulmonary fibrosis [1, 2]. The main type 2 cytokines are interleukin (IL)-13 and IL-4, produced by T helper 2 (Th2) cells and type 2 innate lymphocytes; both are suggested to play a prominent role in fibrosis development [1, 2]. As type 2 immunity is central in the immunopathology of allergic asthma, compounds targeting IL-13 and IL-4 have been developed for asthma [3–6].
European Respiratory Society