Pirfenidone exacerbates Th2-driven vasculopathy in a mouse model of systemic sclerosis-associated interstitial lung disease.
Anna BirnhuberKatharina JandlValentina BiasinElisabeth FließerFrancesco ValzanoLeigh Matthew MarshChristina KrolczikAndrea J OlschewskiJochen WilhelmWolfgang TollerAkos HeinemannHorst OlschewskiMalgorzata WygreckaGrazyna KwapiszewskaPublished in: The European respiratory journal (2022)
This study shows that antifibrotic properties of pirfenidone may be overruled by unwanted interactions with pre-injured endothelium in a setting of high T-helper type 2 inflammation in a model of SSc-ILD. Careful ILD patient phenotyping may be required to exploit benefits of pirfenidone while avoiding therapy failure and additional lung damage in some patients.
Keyphrases
- interstitial lung disease
- idiopathic pulmonary fibrosis
- systemic sclerosis
- mouse model
- pulmonary fibrosis
- rheumatoid arthritis
- oxidative stress
- end stage renal disease
- ejection fraction
- newly diagnosed
- nitric oxide
- dendritic cells
- peritoneal dialysis
- prognostic factors
- regulatory t cells
- immune response
- patient reported outcomes