Gordon Cooke

TL;DR: An innate mechanism for the generation of pulmonary fibrosis, via IL-25 and ILC2, that occurs independently of T-cell–mediated antigen-specific immune responses is presented, suggesting the potential of therapeutically targeting IL- 25 and I LC2 for the treatment of human fibrotic diseases.

TL;DR: It is found that ivacaftor caused marked reductions in sputum P. aeruginosa density and airway inflammation and produced modest improvements in radiographic lung disease in subjects with G551D‐CFTR mutations, raising the possibility that infection and inflammation may progress independently of CFTR activity once cystic fibrosis lung disease is established.

TL;DR: This study reveals the crucial role of defective TLR3 function in promoting progressive IPF and demonstrates increased collagen and profibrotic cytokines inTLR3 knockout mice (tlr3(-/-)) compared with wild-type mice ( tlr3(+/+).

TL;DR: CF neutrophils have a prosurvival phenotype that is associated with an absence of CFTR function and allows increased NET production, which can in turn induce inflammation.

TL;DR: RA-ILD focused research offers the opportunity to identify early asymptomatic disease and define the natural history of this extra articular manifestation, which may provide a unique opportunity to define key regulatory fibrotic events driving progressive disease.