From malignant hypertension to hypertension-MOD: a modern definition for an old but still dangerous emergency

Abstract

The prevalence of malignant hypertension has clearly fallen with the advent of anti-hypertensive medication but has remained stable over the past 30-40 years in spite of progress in diagnosis and management of hypertension. A diagnosis of malignant hypertension is usually based on the association of severely elevated blood pressure with a Keith and Wagener stage III or IV retinopathy. We believe that this definition can be reconsidered for several reasons. Although simple and pragmatic, this definition corresponds to a time when there were few techniques for assessment of hypertensive target organ involvement, and does not take into account involvement of kidney, brain and heart; whereas the overall prognosis largely depends on how much they are affected. On the contrary, the acute blood pressure level and especially diastolic should not be a hard diagnostic criterion as it does not itself constitute the prognosis of the condition. We propose to consider that malignant hypertension with retinopathy is only one of a number of possible presentation(s) of acute hypertension with multi organ damage (hypertension multi organ damage (MOD)) and that the recognition of these hypertensive emergencies, when retinopathy is lacking, be based on acute elevation of BP associated with impairment of at least three different target organs. The objective of a new and expanded definition is to facilitate recognition of these true emergencies. The condition is more common than usually perceived and would have a much worse prognosis than the usual forms of hypertension. Early recognition and management of hypertension-MOD are fundamental to any improvement in prognosis.

Does established vascular kidney disease exist?

“…If left untreated, the 1‐year survival rate has been reported to be only 10%‐20%, with most patients dying within 6 months. Effective treatment has led to a substantial improvement in the survival rate, with the rate at 5 years reaching 60%‐75% in developed countries . Moreover, even after starting renal replacement therapy, mortality rates are higher in nephrosclerosis patients than in other groups (ie interstitial nephropathies)…”

Thrombomodulin and Endothelial Dysfunction: A Disease-Modifier Shared between Malignant Hypertension and Atypical Hemolytic Uremic Syndrome

“…As the prevalence of TMA associated with MHT is 10 times more frequent than that of aHUS, this question deserves further attention [1]. TM has shown promising results in the treatment approach of DIC, with a good safety profile with less bleeding complications in comparison with recombinant human activated protein C [38,53].…”

“…Malignant hypertension (MHT) is a life-threatening condition classically defined by hypertensive retinopathy grade III/IV and/or other end-organ damage, while criteria for blood pressure values are inconsistent but mostly define diastolic blood pressure ≥120 mm Hg [1]. Recent guidelines urge to discriminate between hypertensive emergency and hypertensive urgency.…”

Malignant hypertension as a rare cause of thrombotic microangiopathy

“…However, more modern definitions proposed recently consider the involvement of at least three different target organs as malignant hypertension 2. The target organs typically involved are kidney, heart, brain and microvessels.…”