Vascular Diseases

BENIGN NEPHROSCLEROSIS
glomerulosclerosis
sclerosis of renal
focal ischemia of and chronic
arterioles and
parenchyma tubulointersititial
small arteries
injury

reduction in
renal insufficiency functional renal
mass

renal insufficiency - people with more severe blood pressure elevations, and
diabetes
 Two processes participate in the arterial
lesions:  
• Medial and intimal
thickening,
• Hyaline deposition in
arterioles( hyaline
arteriosclerosis)

• Narrowing of lumen
• Regularly contracted
kidney
 MALIGNANT HYPERTENSION AND
ACCELERATED NEPHROSCLEROSIS
• 1% to 5% of all people with elevated blood
pressure
• systolic pressures greater than 200 mm Hg
and diastolic pressures greater than 120 mm
Hg, papilledema, retinal hemorrhages,
encephalopathy, cardiovascular
abnormalities, and renal failure
• The syndrome is a true medical emergency
requiring the institution of aggressive and
prompt antihypertensive therapy
• 75% of patients survive 5 years, and 50%
survive with restoration of pre-crisis renal
function
 injury causing increased
Chronically
acute exacerbation permeability of the
damaged arterioles
of the hypertension small vessels

fibrinoid necrosis of
arterioles and small
arteries, swelling of Mitogenic factors hyperplastic
the vascular intima, from platelets arteriolosclerosis
and intravascular
thrombosis

self-perpetuating
cycle in which
further narrowing renin-angiotensin
angiotensin II
of the lumens system
causes intrarenal
vasoconstriction,
onion-skinning
• concentrically arranged
smooth muscle cells,
together with fine
concentric layering of
collagen and
accumulation of pale-
staining material that
probably represents
accumulations of
proteoglycans and
plasma proteins.
 RENAL ARTERY STENOSIS
• 2% to 5% of cases of hypertension
• it represents a potentially curable form of
hypertension with surgical treatment
• hypertensive effect, at least initially, is due to
stimulation of renin secretion by cells of the
juxtaglomerular apparatus and the
subsequent production of the vasoconstrictor
angiotensin II.
• most common cause of renal artery stenosis
(70% of cases) is occlusion by an
atheromatous plaque
• second type of lesion leading to stenosis is
so-called fibromuscular dysplasia of the renal
artery
– The lesions may consist of a single well-defined
constriction
• kidney is usually reduced in size and shows
signs of diffuse ischemic atrophy
 THROMBOTIC MICROANGIOPATHIES

• this group of disorders is characterized

clinically by
• Microangiopathic hemolytic anemia,
• thrombocytopenia, and (in many cases)
• renal failure,
• morphologically by thrombotic lesions in
capillaries and arterioles in various tissue
beds, including those of the kidney
 Classification
• Typical HUS
• Atypical HUS
• TTP
• two pathogenetic triggers dominate:
• (1) endothelial injury, and
• (2) platelet activation and aggregation
 Shiga like toxin-
Endothelial injury

reduced endothelial
production of adhesion molecules
prostaglandin I2 and increased production expressed m result in
NO of endothelium- the recruitment of
derived endothelin leukocytes

Platelet aggregation
Promotes thrombosis vasoconstrictiom

TYPICAL HUS
• Vasoconstriction and thrombosis produce
hypoperfusion of kidney- renal failure
• Activation and aggregation of platelets-
platelet consumption – bleeding
manifestaions
• Microangiopathic hemolytic anemia
 Clinical features

• Following a prodrome of influenza-like or

diarrheal symptoms, there is a sudden onset
of bleeding manifestations (especially
hematemesis and melena), severe oliguria,
and hematuria, associated with
microangiopathic hemolytic anemia,
thrombocytopenia, and (in some patients)
prominent neurologic changes.
• Hypertension
 TTP
• Very large vWF multimers can bind platelet
surface glycoproteins and activate platelets
spontaneously,
• ADAMTS13, a plasma protease cleaves vWF
multimers into smaller sizes.
• ADAMTS13 deficiency causes widespread
activation of platelets
• Deficiency is most often caused by
autoantibodies that inhibit ADAMTS13 function.
 the pentad of TTP
• fever, neurologic symptoms,
microangiopathic hemolytic anemia,
thrombocytopenia, and renal failure
• active disease the kidney may show patchy or
diffuse cortical necrosis (described later) and
subcapsular petechiae
• glomerular capillaries are occluded by
thrombi
• arterioles show fibrinoid necrosis