Vascular Pathology (Pathoma)

Question 1

Vasculitis

Answer 1

Inflammation of blood vessel (arteriole wall)

Etiology is usually unknown

Most causes are not infectious

Nonspecific sx and sx of organ ischemia (key to sx of particular vasculititidies). Ischemia from thrombis or fibrosis.

Large vessel (aortic branches) ; medium vessel (muscular arteries) ; or small vessel (arterioles, capillaries, and venules)

Question 2

Temporal (Giant Cell) Arteritis

Answer 2

MC form of vasculitis in older adults (>50); usually females

Branches of carotid artery

Symptoms: Headache (temporal); Visual disturbances (opthalmic); jaw claudication

Flu like sx w/ joint and muscle pain (PMR)

ESR is elevated ( rx if any suspicion before biopsy confirmation)

Question 3

Takayasu Arteritis

Answer 3

Spectrum of Giant Cell (Granulomatous vasculitis in adults classic pt. is young asian female)

Affects major vessels coming off aortic arch.

Sx:
Visual and neurologic symptoms; Weak or absent pulse in an upper extremity (‘pulseless disease’)

ESR is elevated

Rx = steroids

Question 4

Polyarteritis nodosa

Answer 4

Necrotizing vasculitis that can involve any organ except lung.

Presents in young adults w/:
HTN (renal artery); Abdominal pain w/ melana (mesenteric artery); Neurologic disturbances; skin lesions

Associated w/ serum HBsAG

Lesions are present in varying stages. “String of pearls” appearance on imaging. –> transmural fibrinoid necrosis (highlighter pink) and subsequent fibrosis. Combination of fibrosis and small aneurysm (nodes)

Rx: Corticosterois and Cyclophosphamide

Question 5

Kawasaki disease

Answer 5

Classically affects Asian children thrombosis w/ MI or aneurysm w/ rupture.

Rx: Aspirin and IVIG (key ddx –> reye syndrome). Disease is self limited.

Remember kid sitting on motorcycle (palms and soles to drive). Heart goes up while driving motorcycle.

Question 6

Buerger’s Disease

Answer 6

Necrotizing vasculitis involving digits

Presents w/ ulceration, gangrene, and autoampuation of fingers and toes.

Raynaud phenomenon is often present (white –> blue –> red)

Highly associated w/ smoking –> rx = cessation**

Question 7

Weg(C)ener’s Granulomatosis (Granulomatosis w/ polyangitis)

Answer 7

Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys*

Presents w/ middle aged male w/ sinusitis or nasopharyngeal ulceration, hemoptysis w/ bilateral lung infiltrates, and hematuria due to RPGN

Key serum marker = Cytoplasmic anti neutrophil cytoplasmic antibody c-ANCA (levels correlate w/ disease activity)

Biopsy: Large Necrotizing Granulomas w/ adjacent necrotizing vasculitis

Rx: Corticosteroids and Cyclophosphamide. Relapses are common.

Question 8

Necrotizing Polyangiitis

Answer 8

Necrotizing vasculitis involving multiple organs, especially the lung and kidney

Similar to Wegener’s, but no nasopharyngeal involvement or granulomas

Serum perinuclear- anti neutrophili cytoplasmic antibody (p-ANCA) levels correlate w/ disease activity

Rx: Cyclophosphamide and corticosteroids w/ common relapses

Question 9

Churg-Strauss Syndrome

Answer 9

Necrotizing granulmatous vasculitis w/ eosinophils

Involves multiple organs, especially lungs and heart

Asthma and peripheral eosinophilia*

p-ANCA levels correlate w/ disease activity

Question 10

Henoch Schonheim Purpura

Answer 10

Vasculitis due to IgA immune complex deposition

MC vasculitis in children

Presents w/ palpable purpura (bleeding and inflammation) on buttocks and legs, GI bleeding and pain, Hematuria (IgA nephropathy –> IgA in mesangium)

Usually post URI or enteritis (IgA production increased)

Disease is self limited and can recur. Steroids if severe.

Question 11

Systemic HTN

Answer 11

BP >140/90. 20% of US population

Divided into primary or secondary

Question 12

Primary HTN

Answer 12

95% of HTN; no known etiology

Risk factors: Age, race (blacks increased; asians decreased), obesity, stress, lack of physical activity, high salt diet (Na affects both both volume (systolic) and TPR (diastolic)).

Question 13

Secondary HTN

Answer 13

HTN due to identifiable cause.

MC is Renal artery stenosis

Important to rule out.

Question 14

Renal Artery Stenosis

Answer 14

Increased plasma renin –> RAAS –> increased blood volume and TPR ( caused by decreased blood flow hitting JGA), unilateral atrophy of affected kidney.

If bilateral –> ACEI’s contraindicated

Key causes:
Atherosclerosis (elderly males)
Fibromuscular dysplasia (young females)

Question 15

Fibromuscular dysplasia

Answer 15

Cause of secondary HTN often in young females.

Due to congenital defect resulting in irregular thickening of medium sized vessels (especially renal artery)

Strings on peal appearance on CT w/ contrast

Question 16

Benign HTN

Answer 16

Mild or moderate elevation of bp. MC form of

Clinically silent; vessels and organs are damaged over time

Hyaline arteriolosclerosis

Question 17

Malignant HTN

Answer 17

Severe elevation of bp (>200/120)

May arise from preexisting HTN or de novo

Present w/ acute renal failure, headache, and papilledema.

Fibrinoid arteriolosclerosis (Onion skinning)

Medical emergency

Rx: Sodium nitroprusside.

Question 18

Arteriosclerosis

Answer 18

Three pathologic patterns:

1. )Atherosclerosis -thickening of intima* of blood vessel walls. Usually in medium and large vessels.
2. ) Arteriolosclerosis - Thickening of arterioles either due to protein (hyaline) or thickening of smooth muscle (fibrinoid)
3. ) Monckeberg medial sclerosis - Calcification of media of vessel walls. Not super clinically signifcant.

Question 19

Atherosclerosis

Answer 19

Intimal plaque that obstructs blood flow. Mostly consists of necrotic lipid core (cholesterol that can be dystrophically calcified) w/ smooth muscle cap.

Invovles large/medium sized arteries. 4 MC are Abdominal aorta, coronary, popliteal, and internal carotid.

Risk factors:
Modifiable: HTN, Hypercholestemia, smoking, DM

Nonmodifiable: Age, gender (males; estrogen is protective; genetics (family hx is key)

Pathogenesis: Oxidized lipids deposit in intima and are phagocytosed by mphages –> fatty streak (teenagers) –> inflammation and healing –> fibromuscular cap –> narrowed lumen.

Complications: Account for >50% of disease in western world.

Stenosis of medium sized vessels –> peripheral vascular disease; angina; ischemic bowel disease. No sx until >70% stenosis ***

Plaque rupture (at neck) w/ thrombosis; MI, stroke

Plaque rupture w/ embolization. Results in atherosclerotic emboli (hallmark is cholestrol clefts in emboli)

Weakening of vessel wall (thick wall –> ischemia of wall beyond intima)–> aneurysm

Question 20

Arteriolosclerosis

Answer 20

Narrowing of small arteries

Divided into hyaline and hyperplastic types:

Hyaline - caused by proteins leaking into the vessel wall –> thickening –> pink protein deposits on bx. Due to DM (NEG of BM) and benign HTN (pressure pushes proteins in wall)***

Results in reduced vessel caliber w/ end-organ ischemia. Classically produces glomerular scarring. Progresses to CKD ( thus DM and HTN are 2 MCC of CKD).

Presents w/ shrunken kidney and scarring of cortex.

Hyperplastic - involves thickening of smooth muscle creating onion-skin appearance. Due to malignant HTN.*

Results in reduced vessel caliber w/ end organ ischemia as well. May lead to fibrinoid necrosis of vessel wall. Classically causes ARF w/ “flea bitten” appearance (petichiae)

Question 21

Hyaline arteriolosclerosis

Answer 21

Caused by proteins leaking into the vessel wall –> thickening –> pink protein deposits on bx. Due to DM (NEG of BM) and benign HTN (pressure pushes proteins in wall)***

Results in reduced vessel caliber w/ end-organ ischemia. Classically produces glomerular scarring. Progresses to CKD ( thus DM and HTN are 2 MCC of CKD).

Presents w/ shrunken kidney and scarring of cortex.

Question 22

Hyperplastic atherosclerosis

Answer 22

involves thickening of smooth muscle creating onion-skin appearance. Due to malignant HTN.*

Results in reduced vessel caliber w/ end organ ischemia as well. May lead to fibrinoid necrosis of vessel wall. Classically causes ARF w/ “flea bitten” appearance (petichiae)

Question 23

Monkeberg Medial Calcific Sclerosis

Answer 23

Calcification of media; non-obstructive.

Not clinically significant.

Incidental finding on XR or Mammography*.

Question 24

Aortic Dissection

Answer 24

Intimal tear w/ dissection of blood through the media of the aortic wall.

Occurs in proximal 10cm of aorta w/ preexisting weakness of media.

Need both weakness and stress –> HTN is key for both. (HTN can cause hyaline arterilosclerosis of vasovasorum –> weaking of medium).

Also associated w/ inherited defects of connective tissue (marfans and ehlers-danlos)

Presentation is sharp tearing chest pain radiating to the back. MC COD is pericardial tamponade. Can also have obstruction of arch vessels and other aortic arteries (i.e. renal artery). Can also result in fatal hemorrhage.

Question 25

Thoracic aneurysm

Answer 25

Balloon like dilatation of thoracic aorta

Need weakness in aortic wall.

Classically seen in tertiary syphillis (syphillis arteritis of vasovasorm) –> atrophy in wall of blood vessel –> risk for aneursym.

Results in ‘tree-bark’ appearance of aorta due to scarring from end arteritis.

Complications: Dilatation of aortic valve w/ insufficiency. Can also get compression of mediastinal structures. Also rupture and hemorrhage. Can cause get thrombosis or embolus (blood hemostasis and endothelial damage in aneurysm).

Question 26

AAA

Answer 26

Balloon like dilatation of AA. Usually arises below renal arteries, but above aortic bifurcation (lack of vasovasorum in this region).

Primarily due to atherosclerosis (less O2 to tissue w/o vasovasorum) –> atrophy of wall –> weakness –> aneurysm

Classically in a male smoker > 60 w/ HTN.

Present w/ pulsatile mass that grows w/ time.

Major complication is rupture (esp. if >5cm*). Presents w/ triad of hypotension, pulsatile flank mass, and flank pain.

Question 27

Hemangioma

Answer 27

Benign tumor comprised of blood vessels

Commonly present at birth; *often regresses during childhood (don’t remove, esp. on face)

Most often involves skin and liver.

Blanching (vs. bleed into skin)

Question 28

Angiosarcoma

Answer 28

Malignant proliferation of endothelial cells

Highly aggressive

Common sites include skin, breast, and liver

*Liver angiosarcoma is associated w/ exposure to PVC, arsenic , and Thorotrast

Question 29

Kaposi Sarcoma

Answer 29

Low grade malignant proliferation of enothelial cells

Caused by HHV-8

Presents /w purple patches, placques, or nodules on skin (purple due to blood in the endothelial proliferation –> non-blanching!)

May involve visceral organs.

3 patients:
Older eastern european males (surgical resection for rx), AIDS (rx is antiretrovirals –> recover), and Transplant recipients (decrease immunosuppression).