CCN2 and pulmonary fibrosis: Its mechanism specifically involves targeting the von Willebrand factor C domain of the CCN2 protein.[41] Pamrevlumab has been granted US Food and Drug Administration (FDA) Fast Track and Orphan Drug Designation for use in locally advanced pancreatic carcinoma, Duchenne muscular dystrophy, and idiopathic pulmonary fibrosis and has entered into phase 3 clinical trials for these indications.[23] Results of phase 2 trials have thus far shown potential for decreasing tissue fibrosis with functional improvements.[24, 25] No clinical trials to date have noted its effect on bone.