The endothelium as the common denominator in malignant hypertension and thrombotic microangiopathy

• Endothelial dysfunction underlie thrombotic microangiopathies and malignant hypertension.
• Endothelial dysfunction likely promotes hypertension and microangiopathic hemolytic anemia.
• Alternative complement activation may also be important in malignant hypertension and TMA.
• Reducing endothelial injury via complement inhibition beneficial in atypical HUS.
• Clinical trials needed to test targeting endothelium in TTP and malignant hypertension.

The endothelium plays a pivotal role in vascular biology. The endothelium is the primary site of injury in thrombotic microangiopathies including malignant hypertension. Endothelial injury in thrombotic microangiopathies is the result of increased shear stress, toxins, and/or dysregulated complement activation. Endothelial injury can lead to microvascular thrombosis resulting in ischemia and organ dysfunction, the clinical hallmarks of thrombotic microangiopathies. Currently, available therapies target the underlying mechanisms that lead to endothelial injury in these conditions. Ongoing investigations aim at identifying drugs that protect the endothelium.