Vascular Diseases

Question 1

What is the underlying cause of benign nephrosclerosis?

Answer 1

Response to hemodynamic change such as HTN

Question 2

What is the pathogenesis of benign nephrosclerosis?

Answer 2

Sclerosis from medial and intimal thickening with hyaline protein deposition in the arteriolar walls

Question 3

What are some of the morphological findings in benign nephrosclerosis?

Answer 3

Hyaline arteriosclerosis, fibroelastic hyperplasia, ischemic atrophy

Question 4

What is the pathogenesis of malignant nephrosclerosis?

Answer 4

Endothelial cell damage, fibrinoid necrosis of arterioles, onion skinning (duplication of BM)

Question 5

What are the pathologic effects of malignant nephrosclerosis?

Answer 5

Ischemic kidneys, elevated renin via RAAS (makes problem worse).

Question 6

What are the tissue manifestations of malignant hypertension?

Answer 6

Malignant arteriosclerosis/ malignant nephrosclerosis

Question 7

What are the clinical features of malignant hypertension?

Answer 7

BP greater than 200/120
Papilledema
Retinal hemorrhage
Encephalopathy
CV abnormalities
Renal failure
*early symptoms due to ICP

Question 8

What is the most common etiology of large vessel disease related to the kidneys?

Answer 8

Atherosclerosis, with the atheromatous plaques often at the origin of renal artery

Question 9

What is another cause of large vessel disease related to the kidneys that is not atherosclerotic in origin?

Answer 9

Fibromuscular dysphasia (intimal, medial, or adventitial) hyperplasia.

More frequent in young age group, specifically young women.

Question 10

What is thrombotic microangiopathies?

Answer 10

Spectrum of disorders including HUS and TTP that result in an increase in platelet deposition.

Question 11

What are the characteristics of TTP and HUS?

Answer 11

TTP-> thrombocytopenia, microangiopathic hemolytic anemia, thrombosis.

HUS-> TTP w/o the neurologic symptoms

Question 12

What is characteristic of typical HUS?

Answer 12

Diarrheal symptomology from consuming food with bacteria producing shiga-like toxin (E. Coli O157:H7). Mostly in children

Question 13

What are the characteristics of atypical HUS?

Answer 13

• Non-epidemic; non-diarrheal.
• inherited mutations in proteins that regulate complement.
• multiple aquired causes of endothelial injury.

Question 14

What are the characteristics of TTP?

Answer 14

Often associated with inherited or aquired deficiencies of ADAMTS13 which regulates vWF.

Neurologic involvement is prominent

• Fever, neuro issues, thrombocytopenia, microangiopathic hemolytic anemia, renal failure (50% of pts)
• adults

Question 15

What is seen in sickle-cell nephropathy?

Answer 15

Generally hematuria and hyposthenuria
Patchy papillary necrosis
About 30% exhibit proteinuria, usually sub nephrotic