Figure shows the targets of drugs tested in completed clinical trials (red), drugs currently under testing or with a case report (orange), and drugs tested only in preclinical models (blue). For further details, see Table 1 and Table 2. Drugs have been developed to block VEGF-A signaling using neutralizing anti–VEGF-A mAbs (bevacizumab) or soluble trap/decoy receptors that bind VEGF-A (red), VEGF-B (yellow), and PlGF (blue) (aflibercept), or neutralizing anti-VEGFR2 antibodies (D5B1 and DC101). Drugs developed to block intracellular signaling, such as tyrosine kinase receptor inhibitors that block VEGFR2 activity but also other receptors, are currently undergoing testing: pazopanib (VEGFR, PDGFR, c-KIT, and FGFR) and nintedanib (VEGFR, PDGFR, and FGFR). Drugs have also been developed to block PI3K and AKT (VAD044), as well as mTORC1 (sirolimus) and calcineurin and FKBP12 (tacrolimus). Immunomodulatory imide drugs (IMIDS), such as thalidomide and pomalidomide, have been tested in HHT patients. Other drugs have been tested so far only in preclinical models, such as the neutralizing anti-Ang2 monoclonal antibodies (LC10) and inhibitors of CDK4/6 (palbociclib and ribociclib).