Based on the hypothesis that a local activation of a T-cell immune response and upregulation of IFN-γ production has antifibrotic proprieties, we used the HEI3090-positive modulator of the purinergic receptor P2RX7, previously developed in our laboratory (Douguet et al., 2021), to demonstrate that activation of the P2RX7/IL-18 pathway attenuates lung fibrosis in the BLM mouse model. Here, P2RX7 is linked to Bloom syndrome.