Kidney nontumor / medical renal
Vascular disease
Hypertensive renal disease
Malignant hypertension and accelerated nephrosclerosis
Author: Nikhil Sangle, M.D.
Last author update: 3 December 2012
Last staff update: 28 September 2023
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PubMed Search: Malignant hypertension and accelerated nephrosclerosis
Table of Contents
Definition / general | Pathophysiology | Clinical features | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Immunofluorescence description | Electron microscopy descriptionCite this page: Sangle N. Malignant hypertension and accelerated nephrosclerosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneymalignanthyper.html. Accessed April 25th, 2024.
Definition / general
- Also called hypertensive emergency, malignant nephrosclerosis
- Severe hypertension with acute impairment of one or more organ systems (especially CNS, cardiovascular, renal) that may cause irreversible organ damage
Pathophysiology
- Vascular damage (due to chronic hypertension, arteritis, coagulopathy) increases permeability of small vessels to fibrinogen and other plasma proteins
- This causes endothelial injury and platelet deposition, which causes fibrinoid necrosis
- Kidneys become ischemic, which stimulates renin-angiotensin system to produce angiotensin II, which causes renal vasoconstriction, as well as increased aldosterone secretion and salt retention, which elevate blood pressure even further
- May be related to increased TRPC3 expression in vascular endothelium (Dtsch Med Wochenschr 2009;134:2224)
Clinical features
- Usually associated with pre-existing hypertension, glomerulonephritis or reflux nephropathy
- Rarely caused by juxtaglomerular cell tumor (J Med Assoc Thai 2012;95 Suppl 2:S251, J Hypertens 2012;30:974)
- 1 - 5% of patients with hypertension; higher frequency in young men, African-Americans
- Symptoms: diastolic blood pressure 130 mm or more, cardiac symptoms, encephalopathy, headache, nausea, vomiting, loss of consciousness, proteinuria and renal failure
- Treatment: antihypertensive therapy before irreversible renal lesions develop
- Renal survival has improved; mean proteinuria is important prognostic factor (Nephrol Dial Transplant 2010;25:3266)
Gross description
- "Flea bitten" appearance of kidney due to pinpoint petechiae on cortical surface
Gross images
Images hosted on other servers:
Various images
Microscopic (histologic) description
- Fibrinoid necrosis of arterioles, hyperplastic arteriolitis (onion skinning) due to concentric layering of collagen
- May see necrotizing glomerulitis, wrinkling and collapse of capillary walls and small crescents
- Myointimal hyperplasia and hypertropy is associated with acute or persistent severe high blood pressure
- Necrotizing arteriolitis: fibrinoid necrosis of afferent arteriole, often superimposed on hyperplastic or hyaline lesions; media has deposits of deeply eosinophilic and fibrillar material containing fibrin and fibrinogen
- Early changes: profound intimal thickening by myxoid connective tissue, reducing lumen
- Late changes: scarring and concentric thickening of vessel wall by myointimal cells and deposition of basement membrane type material (onion skinning)
Microscopic (histologic) images
Images hosted on other servers:
Various images
Immunofluorescence description
- Occasional fibrin, fibrinogen, IgM and complement
Electron microscopy description
- Swollen endothelium
- May be focally disrupted from glomerular basement membrane by accumulation of electron-dense material
