TGFB1 and systemic sclerosis: This results in the activation of an immune response including cytokine release, up-regulation of pro-fibrotic mediators and recruitment of fibroblasts and fibrocytes.[50] Dysregulated TGFbeta results in fibroblast proliferation and differentiation along with extracellular matrix production and deposition.[51] The WNT/β-catenin pathway has been shown to stimulate fibroblasts to myofibroblasts with suggestions WNT and TGFbeta may create a reciprocal loop sustaining myofibroblast activation.[52,53,54] Derived myofibroblasts are thought to be the key effector cell of fibrosis in SSc-ILD.[55]