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Moderate to severe hypertensive retinopathy and hypertensive encephalopathy in adults

William J Elliott, MD, PhD
Joseph Varon, MD, FACP, FCCP, FCCM, FRSM
Section Editors
George L Bakris, MD
Norman M Kaplan, MD
Deputy Editor
John P Forman, MD, MSc


Hypertensive emergencies are acute, life-threatening conditions resulting from markedly increased blood pressure (BP), generally ≥180/120 mmHg (table 1), characterized by acute, ongoing target-organ damage [1-5]. In addition to the hypertensive emergencies that are discussed in detail elsewhere (see "Evaluation and treatment of hypertensive emergencies in adults"), two less common clinical syndromes induced by acute, severe hypertension include:

Moderate to severe hypertensive retinopathy − Moderate to severe hypertensive retinopathy, corresponding to grades III and IV hypertensive retinopathy, is characterized by retinal hemorrhages, exudates, and papilledema (image 1) [6]. (See "Ocular effects of hypertension", section on 'Ocular diseases directly related to hypertension'.)

Severe hypertensive retinopathy was formerly called "malignant hypertension," a term that clinicians should avoid (although it is used for administrative purposes in the United States). Moderate hypertensive retinopathy was often referred to as "accelerated hypertension," but this term should likewise be avoided. Patients with moderate to severe hypertensive retinopathy frequently have acute hypertensive nephrosclerosis (formerly called "malignant nephrosclerosis"), although kidney biopsies are seldom performed.

Historically, papilledema was the hallmark of a more advanced condition (ie, "malignant hypertension") associated with a higher mortality (akin to having a malignancy). However, the advent of effective antihypertensive drug therapy has improved the prognosis and patients with moderate and severe hypertensive retinopathy have similar outcomes [7]. Thus, acute treatment of such patients is the same whether or not papilledema is present.

Hypertensive encephalopathy − Hypertensive encephalopathy refers to the presence of signs and/or symptoms of cerebral edema caused by severe and/or sudden rises in BP. It is primarily a diagnosis of exclusion after other causes of central nervous system dysfunction are ruled out, and it characteristically responds dramatically to acute lowering of the mean arterial pressure, sometimes by as little as 10 to 15 percent. (See "Evaluation and treatment of hypertensive emergencies in adults", section on 'Neurologic emergencies'.)


With mild to moderate elevations in blood pressure (BP), the initial response is arterial and arteriolar vasoconstriction. This autoregulatory process both maintains tissue perfusion at a relatively constant level and prevents the increase in pressure from being transmitted to the smaller, more distal vessels [8].

With increasingly severe hypertension, however, autoregulation eventually fails (figure 1) [8]. The ensuing rise in pressure in the arterioles and capillaries leads to acute damage to the vascular wall. Disruption of the vascular endothelium then allows plasma constituents (including fibrinoid material) to enter the vascular wall, thereby narrowing or obliterating the vascular lumen. Within the brain, the breakthrough vasodilation from failure of autoregulation leads to the development of cerebral edema and the clinical picture of hypertensive encephalopathy [8].

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Literature review current through: Dec 2017. | This topic last updated: Jul 28, 2016.
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  1. Whelton PK, Carey RM, Aronow WS, et al. 2017 ACC/AHA/AAPA/ABC/ACPM/AGS/APhA/ASH/ASPC/NMA/PCNA Guideline for the Prevention, Detection, Evaluation, and Management of High Blood Pressure in Adults: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Hypertension 2017.
  2. Marik PE, Varon J. Hypertensive crises: challenges and management. Chest 2007; 131:1949.
  3. Mancia G, Fagard R, Narkiewicz K, et al. 2013 ESH/ESC Guidelines for the management of arterial hypertension: the Task Force for the management of arterial hypertension of the European Society of Hypertension (ESH) and of the European Society of Cardiology (ESC). J Hypertens 2013; 31:1281.
  4. Cremer A, Amraoui F, Lip GY, et al. From malignant hypertension to hypertension-MOD: a modern definition for an old but still dangerous emergency. J Hum Hypertens 2016; 30:463.
  5. Adebayo O, Rogers RL. Hypertensive Emergencies in the Emergency Department. Emerg Med Clin North Am 2015; 33:539.
  6. Wong TY, Mitchell P. Hypertensive retinopathy. N Engl J Med 2004; 351:2310.
  7. Ahmed ME, Walker JM, Beevers DG, Beevers M. Lack of difference between malignant and accelerated hypertension. Br Med J (Clin Res Ed) 1986; 292:235.
  8. Strandgaard S, Paulson OB. Cerebral blood flow and its pathophysiology in hypertension. Am J Hypertens 1989; 2:486.
  9. Montgomery HE, Kiernan LA, Whitworth CE, et al. Inhibition of tissue angiotensin converting enzyme activity prevents malignant hypertension in TGR(mREN2)27. J Hypertens 1998; 16:635.
  10. Patel R, Ansari A, Grim CE, Hidaka M. Prognosis and predisposing factors for essential malignant hypertension in predominantly black patients. Am J Cardiol 1990; 66:868.
  11. Davis BA, Crook JE, Vestal RE, Oates JA. Prevalence of renovascular hypertension in patients with grade III or IV hypertensive retinopathy. N Engl J Med 1979; 301:1273.
  12. Vaughan CJ, Delanty N. Hypertensive emergencies. Lancet 2000; 356:411.
  13. Kaplan NM, Victor RG. Hypertensive emergencies. In: Kaplan's Clinical Hypertension, 11th Edition, Lippincott Williams & Wilkins, Philadelphia 2015. p.263.
  14. van den Born BJ, Hulsman CA, Hoekstra JB, et al. Value of routine funduscopy in patients with hypertension: systematic review. BMJ 2005; 331:73.
  15. Phillips SJ, Whisnant JP. Hypertension and the brain. The National High Blood Pressure Education Program. Arch Intern Med 1992; 152:938.
  16. Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996; 334:494.
  17. Kitaguchi H, Tomimoto H, Miki Y, et al. A brainstem variant of reversible posterior leukoencephalopathy syndrome. Neuroradiology 2005; 47:652.
  18. Meylaerts L, Ooms V, Lyra S, et al. Hypertensive brain stem encephalopathy in a patient with chronic renal failure. Clin Nephrol 2006; 65:138.
  19. Sandset EC, Bath PM, Boysen G, et al. The angiotensin-receptor blocker candesartan for treatment of acute stroke (SCAST): a randomised, placebo-controlled, double-blind trial. Lancet 2011; 377:741.
  20. Ledingham JG, Rajagopalan B. Cerebral complications in the treatment of accelerated hypertension. Q J Med 1979; 48:25.
  21. Haas DC, Streeten DH, Kim RC, et al. Death from cerebral hypoperfusion during nitroprusside treatment of acute angiotensin-dependent hypertension. Am J Med 1983; 75:1071.
  22. Woods JW, Blythe WB. Management of malignant hypertension complicated by renal insufficiency. N Engl J Med 1967; 277:57.
  23. Mourad G, Mimran A, Mion CM. Recovery of renal function in patients with accelerated malignant nephrosclerosis on maintenance dialysis with management of blood pressure by captopril. Nephron 1985; 41:166.
  24. Angeli P, Chiesa M, Caregaro L, et al. Comparison of sublingual captopril and nifedipine in immediate treatment of hypertensive emergencies. A randomized, single-blind clinical trial. Arch Intern Med 1991; 151:678.
  25. Grossman E, Messerli FH, Grodzicki T, Kowey P. Should a moratorium be placed on sublingual nifedipine capsules given for hypertensive emergencies and pseudoemergencies? JAMA 1996; 276:1328.
  26. Lip GY, Beevers M, Beevers DG. Does renal function improve after diagnosis of malignant phase hypertension? J Hypertens 1997; 15:1309.
  27. DUSTAN HP, SCHNECKLOTH RE, CORCORAN AC, PAGE IH. The effectiveness of long-term treatment of malignant hypertension. Circulation 1958; 18:644.
  28. van den Born BJ, Honnebier UP, Koopmans RP, van Montfrans GA. Microangiopathic hemolysis and renal failure in malignant hypertension. Hypertension 2005; 45:246.
  29. González R, Morales E, Segura J, et al. Long-term renal survival in malignant hypertension. Nephrol Dial Transplant 2010; 25:3266.