Hypertensive Emergency In UMOD-Related Autosomal Dominant Tubulointerstitial Kidney Disease

Hypertensive emergency is characterized by an acute elevation in blood pressure with evidence of impending or progressive acute target organ damage. Management relies mainly on intravenous medications guided by the type of target-organ damage, but there is considerable variability in practice regarding the choice of medications and optimal therapy. Such variables include the choice of agent and the blood pressure goal, but also underlying medical conditions. We report a case of hypertensive emergency in a 39-year-old-male with a rare genetic condition, UMOD-related autosomal dominant tubulointerstitial kidney disease which gave rise to adolescent gout, worsening kidney function over decades and treatment-resistant hypertension.