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Circulation Part 2 > Vasculities- Bricks > Flashcards

Vasculities- Bricks Flashcards

1
Q

Vasculitis is _

A

Vasculitis is inflammation of the blood vessel walls stimulated by activation of the immune system
* It often manifests in predisposed individuals after an environmental trigger

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2
Q

Vasculities can arise as primary conditions or they can be secondary to conditions such as:

A

Vasculities can arise as primary conditions or they can be secondary to conditions such as hepatitis C, rheumatoid arthritis, lupus

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3
Q

What are some of the consequences of untreated vasculitis?

A

If left untreated, vasculitis can lead to
* Tissue ischemia
* Organ necrosis
* Aneurysms

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4
Q

Some general findings of vasculitides include _

A

Some general findings of vasculitides include fever, malaise, elevated ESR and CRP

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5
Q

Large vessel vasculitis includes _ and _

A

Large vessel vasculitis includes Giant Cell arteris and Takayasu arteritis

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6
Q

Giant cell arteritis most commonly affects _

A

Giant cell arteritis most commonly affects carotid artery branches

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7
Q

Takayasu arteritis involves _ and _

A

Takayasu arteritis involves aortic arch and proximal great vessels
* Including subclavian, carotid, mesenteric, renal arteries
* Sometimes called pulseless disease

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8
Q

A patient with new-onset unilateral severe headache in the context of systemic illness may have _

A

A patient with new-onset unilateral severe headache in the context of systemic illness may have Giant Cell arteritis
* Left untreated, this can progress to blindness

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9
Q

Medium vessel vasculitis includes _ and _

A

Medium vessel vasculitis includes polyarteritis nodosa (PAN) and Kawasaki disease

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10
Q

_ is a medium vessel systemic vasculitis that generally involves skin, renal, and visceral vesssels but spares the lungs

A

PAN is a medium vessel systemic vasculitis that generally involves skin, renal, and visceral vesssels but spares the lungs

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10
Q

The leading cause of chest pain in children comes from _

A

The leading cause of chest pain in children comes from Kawasaki disease
* Most often seen in young children

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11
Q

Small vessel vasculitis causes _ , _ , and _ disease but may involve other organs too; patients sometimes have _ or _ skin findings

A

Small vessel vasculitis causes renal , lung , and skin disease but may involve other organs too; patients sometimes have petechiae or palpable purpura skin findings

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12
Q

5 types of small vessel vasculitis

A
  1. Granulomatosis with polyangiitis (GPA)
  2. Eosinophilic granulomatosis with polyangiitis (EGPA)
  3. IgA vasculitis
  4. Microscopic polyangiitis (MPA)
  5. Anti-glomerular basement membrane disease (anti GBM)
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13
Q

The mechanism behind vasculitides involve _

A

The mechanism behind vasculitides involve immune complex deposition and the presence of:
* Antineutrophil cytoplasmic antibodies (ANCAs)
* Anti-endothelial cell antibodies
* Autoreactive T cells

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14
Q

ANCAs are _

A

ANCAs are a group of antibodies directed against components of neutrophils, monocyte lysosomes, endothelial cells
* The exact role of ANCAs in the pathogenesis of vasculitis is unknown but they are thought to mediate damage through the activation of neutrophils –> which then release proteolytic enzymes and reactive oxygen species

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15
Q

C-ANCAs are _ and they target _

A

C-ANCAs are cytoplasmic ANCAs and they target proteinase 3, a component of neutrophil azurophilic granules

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16
Q

P-ANCAs are _ and they target _

A

P-ANCAs are perinuclear ANCAs and they target myeloperoxidase
* Recall that myeloperoxidase is found in lysosomal granules and forms ROS

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17
Q

Name the histologic features of vasculitis

A

Histologic features of vasculitis include:
* Infiltration of WBC fibrinoid necrosis
* Granuloma formation

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18
Q

Fibrinoid necrosis is a type of tissue death seen when _

A

Fibrinoid necrosis is a type of tissue death seen when antigens and antibodies are deposited in vessel walls –> mediates vessel wall destruction

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19
Q

Fibrinoid necrosis is seen in _ and _ vasculitis

A

Fibrinoid necrosis is seen in PAN and SLE vasculitis

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20
Q

Granulomas are characteristic of four types of vasculitides:

A

Granulomas are characteristic of four types of vasculitides:
1. GCA
2. Takayasu arteritis
3. GPA
4. EGPA

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21
Q

Giant cell arteritis (GCA) classically affects _ population

A

Giant cell arteritis (GCA) classically affects older females (50+)

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22
Q

GCA commonly affects _ arteries; it may also involve the vertebral and opthalamic arteries and cause blindness

A

GCA commonly affects temporal arteries; it may also involve the vertebral and opthalamic arteries and cause blindness
* The branches of the ophthalamic artery that arise from the internal carotid artery are at risk

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23
Q

GCA has a strong associated with an inflammatory condition called _ that causes diffuse pain and stiffness of the shoulders, neck, and hips

A

GCA has a strong associated with an inflammatory condition called polymyalgia rheumatica (PMR) that causes diffuse pain and stiffness of the shoulders, neck, and hips

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24
Q

Giant cell arteritis can be characterized by _ on histology; however it is not required for diagnosis

A

Giant cell arteritis can be characterized by multinucleated giant cells on histology; however it is not required for diagnosis

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25
Q

The definitive diagnosis for giant cell arteritis is _

A

The definitive diagnosis for giant cell arteritis is temporal artery biopsy
* In patients with high suspicion, start treatment right away without biopsy to avoid vision loss

Halo seen on doppler ultrasound

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26
Q

Treatment for giant cell arteritis is _

A

Treatment for giant cell arteritis is high-dose corticosteroids like prednisone

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27
Q

The classic population affected by Takayasu arteritis is _

A

The classic population affected by Takayasu arteritis is younger females of Asian descent
* Age of onset 10-40

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28
Q

Takayasu arteritis presents with _ symptoms

A

Takayasu arteritis presents with generalized symptoms as well as arthralgias, myalgias, claudication, limb pain

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29
Q

The histology of Takayasu is similar to GCA, showing arterial wall inflammation of _ cells

A

The histology of Takayasu is similar to GCA, showing arterial wall inflammation of CD4, CD8, histocytes, macrophages, plasma cells

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30
Q

_ is a complication of Takayasu that occurs because of proximal narrowing of the subclavian artery –> retrograde blood flow into the vertebral artery causes syncope, dizziness, and neuro deficits

A

Subclavian steal syndrome is a complication of Takayasu that occurs because of proximal narrowing of the subclavian artery –> retrograde blood flow into the vertebral artery causes syncope, dizziness, and neuro deficits
* Essentially we take blood flow from the brain to perfuse the arm –> causes lightheadedness

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31
Q

Another complication of Takayasu is vasculitis of the _ arteries which can consequently activate RAAS

A

Another complication of Takayasu is vasculitis of the renal arteries which can consequently activate RAAS
* Renovascular hypertension

32
Q

Patient presents with a bruit of the subclavian or abdominal aorta and has a difference in systolic blood pressure between the arms; this might be _

A

Patient presents with a bruit of the subclavian or abdominal aorta and has a difference in systolic blood pressure between the arms; this might be Takayasu arteritis

33
Q

Treatment for Takayasu arteritis is _

A

Treatment for Takayasu arteritis is corticosteroids

34
Q

Explain the process of immune complex deposition of vasculitis

A

Immune complexes deposit in the walls of vessels –> activates complement –> recruits neutrophils –> neutrophils release lysosomal enzymes which damage the vessel wall
* This occurs in PAN and IgA

35
Q

The body develops antibodies against components of neutrophils, monocyte lysosomes, and endothelial cells called _

A

The body develops antibodies against components of neutrophils, monocyte lysosomes, and endothelial cells called anti-neutrophil cytoplasmic antibodies (ANCAs)

36
Q

How are ANCAs involved in immune-mediated vasculities?

A

ANCAs activate neutrophils –> release proteolytic enzymes and ROS –> this ends up injuring the vasculature

37
Q

C-ANCA targets _ in the PMN azurophilic granules

A

C-ANCA targets proteinase 3 in the PMN azurophilic granules

C stands for cytoplasmic
38
Q

P-ANCA targets _ in the lysosomal granules

A

P-ANCA targets myeloperoxidase in the lysosomal granules

P stands for perinuclear
39
Q

We can also have some anti-endothelial cell antibodies involved in _ types of vasculitis

A

Anti-endothelial cell antibodies:
* GPA
* Kawasaki disease
* Takayasu arteritis

40
Q

Autoreactive T cells can also trigger vascular injury via _

A

Autoreactive T cells can also trigger vascular injury via cytokine production –> macrophage activation –. B cell activation –> antibody formation against SM and endothelial cells

41
Q

The deposition of Ag:Ab complex in the vessel wall leads to a special kind of necrosis called _

A

The deposition of Ag:Ab complex in the vessel wall leads to a special kind of necrosis called fibrinoid necrosis

42
Q

Two defining features of vasculitis include _ and _

A

Two defining features of vasculitis include inflammatory WBCs and fibrinoid necrosis

43
Q

Granulomas are nodules of _ and _ cells; they are characteristic of:

A

Granulomas are nodules of macrophages and giant cells; they are characteristic of:
* GCA
* Takayasu arteritis
* GPA
* EGPA

44
Q

Patient presents with a focal granulmona formation and has impaired vision

A

Patient presents with a focal granulmona formation and has impaired vision –> giant cell arteritis

45
Q

Two stages of takayasu

A

Recall that it is caused by granulomatous inflammation of med-large arteries
1. Inflammatory phase fever, fatigue, weight loss
2. Stenosis effects decreased pulses, claudication

46
Q

The best definitive way to diagnose giant cell arteritis is _

A

The best definitive way to diagnose giant cell arteritis is biopsy

47
Q

We can detect Takayasu arteritis via _ imaging

A

We can detect Takayasu arteritis via MR angio or CT angio

48
Q

Kawasaki disease is idiopathic? or may be caused by _

A

Kawasaki disease is idiopathic? or may be caused by incidental infection with nonspecific immune system activation –> systemic necrotizing vasculitis in small-medium vessels

49
Q

How does Kawasaki disease present?

A

Kawasaki presents with “crash and burn”
* Conjunctivitis
* Rash
* Adenopathy
* Strawberry tongue
* Hand and foot
* Fever for 5 days

50
Q

The danger of kawasaki disease is that about 70% of cases involve the _ arteries

A

The danger of kawasaki disease is that about 70% of cases involve the coronary arteries –> can lead to aneurysm

51
Q

We treat kawasaki disease with _ and _

A

We treat kawasaki disease with IVIG and aspirin

52
Q

The medium vessel vasculitis that affects adults (middle aged) is _

A

The medium vessel vasculitis that affects adults (middle aged) is Polyarteritis Nodosa
* Affects more males than females

53
Q

Explain the pathogenesis of PAN

A

PAN is idiopathic –> type 3 immune response –> necrotizing inflammation of the vessel wall –> fibrinoid necrosis –> thrombosis –> infarct

54
Q

In about 30% of PAN cases, the causative antigen is _

A

In about 30% of PAN cases, the causative antigen is Hepatitis B

55
Q

Classically, PAN affects most organ systems but spares the _

A

Classically, PAN affects most organ systems but spares the lungs

56
Q

The complications of PAN include _

A

The complications of PAN include renal failure, MI, stroke

57
Q

The classic population affected by GPA (granulomatosis with polyangiitis) is _

A

The classic population affected by GPA (granulomatosis with polyangiitis) is white middle aged men

58
Q

Importantly, the pathogenesis of GPA involves _

A

Importantly, the pathogenesis of GPA involves c-ANCA

59
Q

The triad of symptoms in GPA include:

A
  1. Respiratory tract- saddle nose deformity
  2. Lung- infiltrates or cavities causing dyspnea, hemoptysis
  3. Kidney disease- nephritic syndrome and possible glomerulonephritis
60
Q

GPA may present with _ skin findings

A

GPA may present with petechia, pupuric skin lesions

61
Q

Eosinophilic granulomatosis with polyangiitis (EGPA) typically present in _ population

A

Eosinophilic granulomatosis with polyangiitis (EGPA) typically present in adults aged 40-50

62
Q

Some of the EGPA cases will be positive for _

A

Some of the EGPA cases will be positive for p-ANCA (30-40%)

63
Q

Three stages of progression in EGPA

A
  1. Allergic rhinitis/ asthma- or may be sinusitis, nasal polyps
  2. Eosinophilic stage- high eosinophil count; the lungs and the GI tract are involved –> we have weight loss, fever, malaise, GI bleeding, abdominal pain
  3. Vasculitic stage- severe blood vessel inflammation –> ischemia to the GI system and heart
64
Q

How does IgA vasculitis normally present?

A

IgA vasculitis most commonly occurs in children following a URI –> IgA immune complexes, fibrin, and complement deposits in small blood vessels

65
Q

Clinical presentations of IgA vasculitis

A

IgA vasculitis presents as:
* Abdominal pain
* Arthritis
* Glomerulonephritis
* Petechiae/ purpura (on the bum)

66
Q

How do we treat IgA vasculitis?

A

IgA vasculitis is usually self-limiting; in severe cases of renal disease we can treat with corticosteroids

67
Q

Microscopic polyangiitis is another small vessel vasculitis that is associated with _

A

Microscopic polyangiitis is another small vessel vasculitis that is associated with p-ANCA

68
Q

MPA can affect multiple organs but the classic triad includes _ , _ , and _

A

MPA can affect multiple organs but the classic triad includes lungs , kidneys , and skin
* Lungs- hemoptysis
* Kidneys- hematuria, proteinuria, hypertension
* Skin- palpable purpura

69
Q

Mixed cryoglobulinemia syndrome occurs when _

A

Mixed cryoglobulinemia syndrome occurs when Ig precipitates at cold temperatures and affects the small vessels

70
Q

The most common associated pathogen with mixed cryoglobulinemia syndrome is _

A

The most common associated pathogen with mixed cryoglobulinemia syndrome is hepatitis C
* HBV
* HIV
* Bacteria
* Fungi
* SLE
* RA

71
Q

Mixed cryoglobulinemia syndrome presents with many of the same symptoms as the other small vessel vasculitides; however, the classic triad is _

A

Mixed cryoglobulinemia syndrome presents with many of the same symptoms as the other small vessel vasculitides; however, the classic triad is
1. Arthralgias
2. Palpable purpura
3. Weakness

Other symptoms: peripheral neuropathy, liver disease, renal disease, raynauds

72
Q

Goodpasture’s syndrome is another name for _

A

Goodpasture’s syndrome is another name for anti-glomerular basement membrane disease
* Caused by autoantibodies directed against collagen epitopes in the kidney and lung glomerular/alveolar basement membranes
* Associated with declining renal function and pulmonary hemorrhage

73
Q

Describe the pathophysiology of thromboangiitis obliterans

A

Thromboangiitis obliterans involves acute inflammation of the small-medium arteries of distal limbs –> thombosis –> infarcts –> raynauds, gangrene, etc
* Most commonly caused by smoking!

74
Q

The classic population to be affected by thromboangiitis obliterans is _

A

The classic population to be affected by thromboangiitis obliterans is 30-50 year old male who smokes

75
Q

Raynauds can occur as a primary or secondary phenomenon; it is ultimately a _ in the skin

A

Raynauds can occur as a primary or secondary phenomenon; it is ultimately a arterial/arteriolar vasospasm in the skin
* Fingers, toes

76
Q

Raynauds is most classically associated with _

A

Raynauds is most classically associated with SLE, scleroderma

77
Q

How does raynauds present?

A

Finger transition from pallor –> blue –> red
* cold, paresthesia, pain

78
Q

We can treat Raynaud with _ , _ , _

A

We can treat Raynaud with Ca2+ channel blockers, alpha blockers, vasodilators

Circulation Part 2 (15 decks)

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