Herein, we demonstrate that antiapoptotic BCL-2 family members are expressed by fibroblasts in both human and murine progressive fibrotic lung disease, that these fibroblasts are highly primed, and that treatment with ABT-263 induced fibroblast apoptosis and reduced fibrosis in 2 preclinical models of PF-ILD. Here, BCL2 is linked to pemphigus foliaceus.