Receptor inhibition with pertussis toxin (PTX) or CCR7 gene silencing with siRNA is able to block CCL21-mediated pathway activation and inhibit functional responses of IPF fibroblasts; thus, CCL21-CCR7 axis-dependent activation of IPF fibroblasts is a promising strategy for IPF treatment [62]. Here, CCL21 is linked to idiopathic interstitial pneumonia.