Vasculitis

Question 1

What type of arteritis is polyarteritis nodosa and what type of arteries (size) does it affect?

Answer 1

this is a necrotizing arteritis that affects medium (muscular) arteries, which supply organs.

Question 2

What vasculitis can induce HTN, syncope, or TIA strokes?

Answer 2

Takayasu’s vasculitis.

Question 3

What types of vessels do giant cell arteritis affect?

Answer 3

Large and medium sized extracranial branches of carotid artery that affects temporal artery, ophthalmic artery, and arteries that go to jaw.

Question 4

What is palpable purpura and what is the cause?

Answer 4

Palpable purpura are raised (palpable) bruises duue to exdtravasation of RBC outside of blood vessels near the dermis that indicate systemic vasculitis. This is caused by nuetrophilic infiltration of vessels that supply the dermis. As a result of damage, blood escapes.

Question 5

What are the 4 large and medium vessel vasculitis?

Answer 5

Large vessel vasculitis:

1) Giant cell (temporal) Arteritis
2) Takayasu’s Arteritis

Medium vessel vasculitis

1) Polyarteritis nodosa
2) Kawasaki Disease

Question 6

What is also known as “pulseless disease” and why?

Answer 6

Takayasu’s arteritis. This is because primary vessels affected are aortic arch and subclavian arteries that may lead to asymmetric BP, bruits, and diminished pulses at the extremities.

Question 7

What is the pathogenesis of Giant cell arteritis?

Answer 7

Something causes CD4+ T-cell mediated immune response that results in a granulomatous vasculitis.

Question 8

Presence of Hepatitis B antigen is indicative of what type of vasculitis?

Answer 8

Polyarteritis nodosa

Question 9

What vasculitis commonly affects Asian children less than 4 years old, and what size vessel does this affect?

Answer 9

Kawasaki Disease, which affects medium sized vessels.

Question 10

Tx of Kawasaki Disease

Answer 10

IVIG and ASA. Aspirin is an anti-platelet, decreasing risk for thrombus formation.

Question 11

What are the main differences between giant cell arteritis and takayasu’s arteritis in regards to age at onset, ethnic ancestry, course, and vessels?

Answer 11

1) Age at onset: Giant cell arteritis occurs in pts older than 50 while Takayasu’s occurs in pts younger than 40
2) Ethnic ancestry: Giant cell arteritis affects northern european pts mostly while Takaytasu’s affects young japanese women.
3) Course: Giant cell arteritis is self-limited (however should still give prednisone due to risk of blindness) while Takaysu’s arteritis is more chronic even with therapy.
4) Vessels affected: Giant cell arteritis affects branches of carotid arteries (e.g. temporal + ophthalmic) while Takayasu’s arteritis affects aortic arch and main branches, subclavian.

Question 12

What are the 2 general clinical features of all types of vasculitis?

Answer 12

1) Constitutional sx of inflammation: Fever, rash, myalgia, fatigue, athralgia, malaise, weight loss
2) Sx of vascular injury that result in organ ischemia/infarction due to:
- Fibrosis and narrowing of lumen: Inflammation of BV wall heals, resulting in fibrosis, narrowing of lumen and subsequent decreased blood flow to organ.
- Thrombosis: Inflammatory response results in endothelial damage, which exposes subendothelial collagen + tissue factors, which activates coagulation cascade and forms thrombus

Question 13

What are the sx of Kawasaki’s disease?

Answer 13

1) Unremitting fever (>5 days) thats resistant to antipyretics
2) Conjunctivitis
3) Erythematous rash of palms and soles with desquamation
4) Cervical lymphadenopathy
5) Changes in lips and oral cavity (dry, cracked lips + strawberry tongue)

Question 14

Which vasculitis needs a large segment of a vessel for diagnosis and why?

Answer 14

Need large segment of artery to diagnose Giant cell arteritis. This is because the lesions are segmental and therefore need to make sure to get large enough piece to pick up the disease. Negative biopsy does not exclude disease bc may have only taken piece of vessel w/o the lesions. Diagnosis require at least 1 cm of artery

Question 15

What type of vasculitis is most common among young asian females (age 25-35)?

Answer 15

Takayasu’s arteritis

Question 16

Which layer(s) of elastic lamina are affected by polyarteritis nodosa and what does this make the vessels susceptible to?

Answer 16

Both internal and external elastic lamina. This weakens the arterial wall, which can lead to aneurysm or rupture.

Question 17

What is ESR? Which vasculitis has an elevated ESR (>100)?

Answer 17

Indirect measure acute phase reaction (proteins that build up in blood after inflammatory response). ESR is above 100 in Giant Cell arteritis patients (+ Takayasu’s arteritis)

Question 18

What vasculitis presents with physical exam findinsg such as scalp tenderness and nodularity/tenderness or absent pulsations of temporal artery?

Answer 18

Giant cell arteritis

Question 19

What is Takayasu’s arteritis?

Answer 19

Granulomatous arteritis that involves aortic arch, main branches, or pulmonary artery.

Question 20

Most common form of vasculitis in pts OVER 50

Answer 20

Giant cell arteritis

Question 21

What occurs during the acute vs. late phase of polyarteritis nodosa?

Answer 21

Acute phase: transmural inflammation of BV wall with fibrinoid necrosis. This makes arterial walls weak, which can lead to aneurysm.

Late phase: Healing of fibrinoid necrosis leads to massive fibrosis of the wall, creating a “hard” nodule. and narrowing the lumen.

Question 22

What is the prognosis of pts wiht polyarteritis nodosa?

Answer 22

It is fatal if untreated. 80% 5 year survival rate if treated.

Question 23

What is giant cell arteritis?

Answer 23

granulomatous vasculitis involving branches of carotid arteries.

Question 24

What occurs in response to granulomatous inflammation in the media in regards to blood flow, and what large artery vasculitis has this type of rxn?

Answer 24

In response to granulomatous inflammation in the media, the lumen of the vessel narrows, limiting blood flow to the target areas. This is seen in Takayasu’s and Giant cell arteritis.

Question 25

How does polyarteritis nodosa affect the lumen of the vessels and why?

Answer 25

During the late phase, healing of inflammatory process occurs, which causes massive fibrosis and formation of a node. This narrows the lumen of the BV.

Question 26

Where do lesions from polyarteritis nodosa occur in the medium sized arteries?

Answer 26

They preferentially occur at the branch points

Question 27

Age group affected for polyarteritis nodosa

Answer 27

Adults 45-65 (middle aged)

Question 28

What are the histolopathological/ biopsy findings in giant cell arteritis?

Answer 28

1) Giant cells (multinucleated cells of macrophages)
2) Intimal thickening/ fibrosis
3) Elastic lamina fragmentation
4) Granulomatous inflammation in the media

Question 29

What is the most important factor in treating giant cell arteritis and why?

Answer 29

If giant cell arteritis is suspected, need to start pt on immediate tx on corticosteroids (e.g. prednisone) even w/o confirmation via biopsy results This is because patients can develop thrombosis of opthalmic artery, leading to optic nerve ischemia, which can lead to blindness (irreversible).

Question 30

What is vasculitis?

What are the 3 main mechanisms of vasculitis for small, medium, and large vessels?

Answer 30

Vasulitis is an immune-mediated (inflammatory) destruction of large, medium, and/or small BVs.

Mechanisms:

1) T-cell response with granuloma formation
2) Immune (Antibody) complex deposition into vasculature
3) Anti-Neutrophil Cytoplasmic Antibodies (ANCA)

Question 31

What are the sx of Takayasu’s arteritis?

Answer 31

1) Constitutional sx: fatigue, malaise, weight loss, night sweats, etc.
2) Asymmetrical blood pressure in extremities, bruits, diminished pulses
3) Extremity claudication (pain w/ use) and coldness/numbness of fingers
4) Visual/ neurologic sx.

Question 32

What specific artery does Kawasaki disease preferentially affect and what is this a concern for?

Answer 32

It primarily affects the coronary arteries, which leads to risk for 1) thrombosis with MI and 2) anuerysm with rupture. This is the #1 cause of acquired heart disease in children.

Question 33

What is the prognosis of Kawasaki’s disease?

Answer 33

Self-limited but in some case, if left untreated, children will develop coronary artery disease that with aneurysm/thrombus foramtion, which may lead to MI.

Question 34

Which organs are affected/ not affected in polyarteritis nodosa and why?

Answer 34

These affect medium arteries which supply organs; thus, they affect multiple organs. However, because they do not affect small vessels such as arterioles, capillaries, and veins, there is no pulmonary involvement or kidney involvement (glomerulonephritis)

Question 35

Tx of polyarteritis nodosa (mild vs. moderate/severe)

Answer 35

Mild: Glucocorticoids

Moderate/severe: Glucocorticoids + Cyclophophamide (immunosuppressive drug)

Question 36

Which layers of the artery are affected in Giant cell arteritis?

Answer 36

all layers of artery

Question 37

What type of vasculitis is Beurger’s disease?

Answer 37

Necrotizing vasculitis

Question 38

Sx of polyarteritis nodosa

Answer 38

1) Constitutional sx (sysytemic inflammation)
2) Neurological sx- asymmetric that affect sensory or motor functions (due to stenosis of vasa nervorum)
3) Skin lesions (e.g. palpable purpura, livedo retcularis)
4) HTN (renal artery stenosis)
5) Abdominal pain and melena (mesenteric artery involvement)

Question 39

Which artery is most commonly affected in polyarteritis nodosa?

Answer 39

Renal arteries (actual kidneys are not affected bc this vasulitis does not affect small BVs)

Question 40

What type of neurological deficits occur with pts with polyarteritis nodosa and why?

Answer 40

Motor or sensory deficits that are asymmetric (may have decreased reflexes). This is because nerves are supplied by vessels called vasa nervorum. These are muscular arteries that are also affected by polyarteritis nodosa. As a reuslt, these nerves do not receive blood supply and get nerve ischemia.

Question 41

What is the pathogenesis of Kawasaki disease?

Answer 41

Macrophages bring antigens into arterial wall, bringing in PMN (e.g. neutrophils) and there is a robust inflammatory response in the blood vessel wall. This leads to intimal and medial destruction, increasing risk for aneurysms and once the artery heals, it can increase risk for thrombosis and MI.

Question 42

What vasculitis involves the digits, presenting with ulceraiton, gangrene, and autoamputation of fingers and toes?

Answer 42

Buerger Disease

Question 43

What is the pathophysiology of Takayasu Arteritis?

Answer 43

Same as Giant cell arteritis. It involves T-cell mediated inflammatory response that results in granulomatous vasculitis with giant cells, with thickening and fibrosis of the tunica intima.

Question 44

Cause and tx of Buerger’s disease

Answer 44

Associated with heavy smoking; tx is smoking cessation.

Question 45

Sx of Giant Cell arteritis

Answer 45

1) Age >50
2) Constitutional sx
3) Headache (temporal artery involvement)
4) Visual symptoms (ophthalmic artery involvement)
5) Jaw claudication (arteries to jaw involvement)

Question 46

What vasculitis is “string of pearls” appearance seen on imaging and why?

Answer 46

Polyarteritis nodosa. This is because the lesions present at differing stages. The early lesions have transmural infalmmation of the BV wall with fibrinoid necrosis. This weakens the walls and increases the risk for aneurysms. However, with healing there is massive fibrosis at the walls creating nodes. This alternating early lesions (with aneurysms) and late lesions seen in a vessel causes the string or pearls appearance on imaging.

Question 47

What is the etiology of all types of vasculitis?

Answer 47

Etioloy is usually unknown; most cases are not infectious.

Question 48

Histopathological findings in Takayasu’s arteritis

Answer 48

Same as giant cell arteritis- giant cells, inflammed vessel walls, intimal fibrosis/thickening. Cannot distinguish Takayasu’s from giant cell arteritis histologically.