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Cardio All > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q
A
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2
Q

Vasculitis ​Conditions of the Aorta

A
  1. Temporal aka Giant Cell
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3
Q

Vasculitis ​Conditions of the Medium Arteries

A
  1. Giant Cell aka Temporal
  2. Polyarteritis Nodosa
  3. Granulomatous w/ polyangiitis
  4. Churg-Strauss
  5. Buerger’s Disease
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4
Q

Vasculitis ​Conditions of the Small Arteries

A
  1. Polyarteritis Nodosa
  2. Granulomatous w/ polyangiitis
  3. Churg-Strauss
  4. Buerger’s Disease
  5. Leukocytoclastic Vasculitis
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5
Q

Vasculitis ​Conditions of the Veins

A
  1. Buerger’s Disease
  2. Leukocytoclastic Vasculitis
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6
Q

Vasculitis ​Conditions of the Caps

A

Leukocytoclastic Vasculitis

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7
Q

Temporal aka Giant Cell

  • Cells
  • Notable Features
  • PT History
  • Vessel Type
A

Temporal aka Giant Cell

  • Cells
    • (+)
      • Lymphocytes
      • MACS
    • Rare
      • Neutrophils
      • Eosinophils
  • Notable Features
    • Can be + or - for granulomas and thrombosis
    • Giant cells not req.
  • PT History
    • Older than 40
    • PT can be +/- polymyalgia rheumatica
  • Vessel Type
    • Aorta
      *
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8
Q

Polyarteritis Nodosa

  • Cells
  • Notable Features
  • PT History
A
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9
Q

Name the condition described below…
Segmental thrombosing, acute and chronic inflammation of medium and small sized arteries. Mainly the tibial and radial arteries.

Almost exclusively in heavy tobacco smokers and typicallybefore 35 y of age.

A

Thromboangiitis Obliterans

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10
Q

Name the condition described below…
Early manifestations include Raynaud, Instep claudication, and superficial nodular phlebitis.

Chronic extremity ulceration may develop.

A

Thromboangiitis Obliterans

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11
Q

What is the described condition…
Typical PT is a middle aged man (although others can be affected). Present with bilateral pneumonitis with nodules and cavitation lesions.
They will also have some degree of renal disease.

A

Granulomatous with Polyangiits

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12
Q

Kawasaki disease is believed to be a ____________________ response directed against cross reactive vascular antigens.

A

Delayed-type hypersensitivity.

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13
Q

Which ANCA is involved with Granulomatosis with Polyangiitis?

A

Wen’C’ers disease PR3-ANCA

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14
Q
A

Granulomatosis with Polyangiitis

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16
Q

How dangerous is Granulomatosis with Polyangiitis, and what is the Tx?

A
  • Mortality rate is 80% if untreated
  • Treatment: Steroids, Cyclophosphamide, TNF inhibitors and Anti-C-cell antibodies
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17
Q
A

Microscopic Polyangiitis

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18
Q

What condition is often marked by WEAKENING OF PULSES in upper extremities?

A

Takayasu

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20
Q

What Vasculitis is an ALLERGIC granulomatosis?

A

Churg-Strauss syndrome

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21
Q

Describe how ANCAs lead to EC injury?

A
    1. Directly activate neutrophils
    1. Stimulates release of reactive oxygen species
    1. Proteolytic enzymes
    1. This leads to EC injury
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22
Q

What is the described condition…
Necrotizing granulomas of the upper respiratory tract and necrotizing, often crescentic, glomerulonephritis.

A

Granulomatosis with Polyangiitis

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23
Q

What is the described condition…
Initially signs and symptoms are non specific, constitutional.

Progression to reduced upper extremity blood pressure, pulse strength and neurological deficits. Ocular disturbances, including visual field defects, retinal hemorrhages and total blindness.

A

Takayasu Arteritis

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24
Q

Other than the temporal arteries, what are the three most common arteries affected by giant cell arteritis?

A

Vertebral arteries
Ophthalmic arteries
Aorta

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25
Q

If you find Vasculitis in just the medium size vessels (arteries), what are the two types of disorders you are thinking and what are the examples?

A

Immune Complex Mediated: Polyarteritis Nodosa

Anti-Endothelial cell antibodies : Kawasaki

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26
Q

What is the described condition…

Likely initiated as a cell-mediated hypersensitivity response to inhaled infectious or environmental antigens.
PR3-ANCA is present in almost 95% of cases and probably drive the tissue injury.

A

Granulomatosis with Polyangiits

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27
Q

What ANCA is associated with Churg-Strauss syndrome?

A

MPO-ANCA

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28
Q
A

Thromboangiitis Obliterans

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29
Q

What term is described:
A small irculating antibody that reacts with neutrophil cytoplasmic antigens, common in many patients with Vasculitis

A

Anti-Neutrophil Cytoplasmic Antibodies (ANCAs)

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30
Q

What is the described condition…

The classic presentation involves some combination of rapidly accelerating hypertension due to renal artery involvement; abdominal pain and bloody stools caused by GI lesions. There will also be diffuse muscular aches and pains, and peripheral neuritis (prominently motor nerves).

A

Polyarteritis nodosa

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32
Q

What is the described condition…
Rare before 50
Constitutional symptoms with facial pain or HA.
Often present with diplopia to vision loss.

What is treatment?

A

Giant Cell Arteritis

Steroids or Anti-TNF therapies

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33
Q

Which ANCA is a ‘Neutrophil Azurophilic Granule Constituent’?

A

Anti-proteinase-3 PR3-ANCA

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34
Q

What is the described condition…

Chronic inflammatory disorder, typically with granulomatous inflammation that principally affects Large to small size arteries in the head?

A

Giant cell (temporal) arteritis

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35
Q

What is the 1st line treatment for Kawasaki Disease?

A

IVIG & aspirin

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36
Q

What is Tx of Microscopic Polyangiitis (2)?

A

Immunosuppressive and removal of offending agent.

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37
Q

What vasculitis that we learned about often leads to cardiomyopathy?

A

Churg-Strauss Syndrome

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38
Q

What is important about the diagnosis of Giant cell arteritis ?

A

This disorder can cause sudden blindness, so hurry it up

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39
Q
A

Giant Cell Arteritis

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40
Q

What is the described condition…

Kidney, heart, liver, and GI vessels are affected in decreasing order of frequency. Impaired perfusion leads to ulceration, infarct, ischemic atrophy or hemorrhage.

A

Polyarteritis Nodosa

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41
Q

What vasculitis disorder do you think of when you think of heavy tobacco smokers under 35?

A

Thromboangiitis Obliterans.

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42
Q

What is the described condition…

In many cases, antibody responses to antigens such as drugs, microorganisms or tumor proteins have been implicated.
Most are associated with MPO-ANCA.
Recruitment and activation of neutrophils in the vascular bed is likely responsible for the disease manifestations.

A

Microscopic polyangiitis

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44
Q

If you find Vasculitis strictly in the LARGE vessels, what conditions are you thinking?

A

Granulomatous diseases (Giant Cell Arteritis and Takayuki Arteritis)

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45
Q

What is the most common form of Vasculitis among older adults in developed countries?

A

Giant Cell Arteritis

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46
Q

What is the described condition…
Likely occurs as a result of a T-Cell mediated Immune response to a uncharacterized vessel wall antigen.
Pro inflammatory cytokines (especially TNF) and anti-EC antibodies may also contribute/

A

Giant Cell Arteritis

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47
Q

What is the described condition…

Clinical Presentation: Hemoptysis, Hematuria, Proteinuria, Abdominal Pain, Muscle plain and palpable cutaneous purpura.

A

Microscopic polyangiitis

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48
Q

Describe how Autoreactive T-Cells cause Vasculitis.

A

They cause injury though the formation of granulomas

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49
Q

What is the described condition…

Acute, febrile, typically self-limiting illness of infancy and childhood associated with an arteritis of mainly large to medium sized vessels.
It’s clinical significance stems from the involvement of coronary arteries.

A

Kawasaki Disease

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50
Q

Name the condition described below…

Rare Disorder
Cutaneous involvement (with palpable purpura), GI bleeds and renal disease.
Cytotoxicity produced by the myocardial eosinophilia infiltrates often leads to cardiomyopathy

A

Churg-Strauss Syndrome

51
Q

What are the two most common/important ANCAs? What are they typically associated with?

A

Anti-proteinase-3 (PR3-ANCA) [previously called c-ANCA]: Associated with ‘Granulomatosis with Polyangiitis’.

Anti-myeloperoxidase (MPO-ANCA) [previously called p-ANCA]: Typically associated with ‘Microscopic Polyangiitis’ and ‘Churg-Strauss syndrome’.

52
Q

Where does takayasu arteritis mainly affect?

A

Aortic arch and arch vessels (pulmonary arteries are involved in 50% of pts)

53
Q

What is the ‘triad’ of Granulomatosis with Polyangiitis?

A
54
Q

What ANCA is associated with granulomatosis with polyangiitis?

A

Anti-Proteinase-3 (PR3-ANCA)

55
Q

What ANCA is associated with granulomatosis with polyangiitis?

In the case of Polyarteritis Nodosa, answer the following questions…

  • (T/F) It is typically episodic, with long symptom-free intervals.
  • (T/F) It is primarily a disease of young adults, but can occur in all age groups, including those over 50.
A

T
T

56
Q

What is the described condition…
Presents like Giant Cell Arteritis but pt is under 50.

A

Takayasu arteritis

57
Q

Untreated, Polyarteritis Nodosa is typically fatal, what organ(s) does it affect mainly to inflict death?

A

Kidneys

58
Q

What are the two organs you think of when you think of Microscopic Polyangiitis?

A

Lungs and kidneys.

59
Q

What is the described condition…

Systemic Vasculitis of small or medium sized muscular arteries, typically involves renal and visceral vessels, but spares the pulmonary circulation.
Most have Hep B too.

A

Polyarteritis nodosa

60
Q

What ANCA is a lysosomal granule constituent involved in oxygen free radical generation?

A

MPO-ANCA

61
Q
A

Takayasu Arteritis

62
Q

What is important to remember about the morphology of Giant Cell Arteritis?

A

It is very patchy.
The involved arterial segments exhibit NODULAR INTIMAL THICKENING that can reduce vessel diameter and cause distal ischemia.

63
Q

Is systemic lupus erythematous a small vessel Vasculitis or a large vessel Vasculitis?

A

Small

64
Q

Name the condition described below… Prevelence in certain ethnic groups (Israeli , Indian subcontinent, and Japanese) and mainly in tobacco smokers, and before 35 y/0.

A

Thromboangiitis Obliterans

65
Q

What is the described condition…
Manifests with Transmural scarring and thickening of the aorta, particularly the aortic arch and great vessels, with severe luminal narrowing of the major branch vessels.

A

Takayasu Arteritis

66
Q
A

Polyarteritis Nodosa

68
Q

What ANCA is associated with microscopic polyangiitis?

A

MPO-ANCA

69
Q
A

Giant Cell Arteritis

70
Q

What is the described condition…

Manifests with conjunctival and oral erythema, blistering, edema of hands and feet, erythema of palms and soles, a desquamative rash and cervical lymph node enlargement.

A

Kawasaki disease

71
Q

What is the described condition…

Necrotizing Vasculitis that generally affects capillaries as well as small ARTERIOLES and venules.

A

Microscopic Polyangiitis

72
Q

What is an example of an Anti-Endothelial Cell antibody caused disorder?

A

Kawasaki

74
Q

(T/F): Giant Cell arteritis can lead to sudden an permanent blindness.

A

True

75
Q

How do you reach a diagnosis of Giant Cell Arteritis?

A

Biopsy of temporal arteries

76
Q

What are the key points of Vasculitis?

A
  • Damage to vessels
  • Every organ system ‘syndrome’ can be involved, or single organ
  • ‘Polyangiitis’ usually denotes small vessel
  • Rare (delays dx)
  • Primary or secondary to another disease
  • Presentation: varies from minimal to life-threatening
77
Q

Describe Primary Vasculitis

A
78
Q

Describe Secondary Vasculitis

A
79
Q

Describe Small Vessel Vasculitis

A
  • Cutaneous post-capillary venules- palpable purpura
  • Glomerular capillaries- haematuria, red cell casts in urine, proteinuria, decline in renal function
  • Pulmonary capillaries- lung hemorrhage
80
Q

Describe Medium Vessel Vasculitis

A

Polyarteritis Nodosa

Kawasaki

81
Q

Describe Medium Vessel Vasculitis

A

Polyarteritis Nodosa

Kawasaki

82
Q

What are the important questions to ask yourself before a case?

A
83
Q

Describe Secondary Vasculitis: Cryoglobulinemic vasculitis

A
84
Q

Things to look for in DX of microscopic polyangiitis

A
  • protein in urine
  • normal kidneys
  • Renal biopsy: “crescentic glomerulonephritis”
  • P Anti-neutrophil cytoplasmic antibody positive (Anti-MPO titre 504)
  • ANCA associated vasculitis–microscopic polyangiitis (MPA)
85
Q

Things to look for in DX of microscopic polyangiitis

A
  • protein in urine
  • normal kidneys
  • Renal biopsy: “crescentic glomerulonephritis”
  • P Anti-neutrophil cytoplasmic antibody positive (Anti-MPO titre 504)
  • ANCA associated vasculitis–microscopic polyangiitis (MPA)
86
Q

Things to look for in DX- granulomatosis with polyangiitis (wegeners)

A
87
Q

In Small vessel vasculiitis- Granulomatosis with polyangiitis (Wegeners), what clinical features do expect to find?

A
88
Q

How do you DX- Churg-Strauss syndrome?

A
89
Q

What are the clinical features of Churg-Strauss syndrome?

A
90
Q

What are the clinical features of Churg-Strauss syndrome?

A
91
Q

Describe the Mononeuritis Multiplex

A
  • ● Mononeuritis multiplex
    • ○ Peripheral neuropathy–asymmetric or symmetric ○ Seen in up to 45% of patients with EGPA (Churg-Strauss)
    • Causes
      • ■ Leprosy (most common)
      • ■ DM
      • ■ Vasculitis
      • ■ Sarcoidosis
      • ■ Amyloidosis
      • ■ Malignancy
      • ■ Neurofibromatosis
      • ■ HIV infection
      • ■ Idiopathic multifocal motor neuropathy
92
Q

Dx this

A
93
Q

Dx this

A
94
Q

Dx this

A
95
Q

Dx this

A
96
Q

Dx this

A
97
Q

What does this describe?​

A

IgA vasculitis (henoch-schonlein)

98
Q

What does this describe?​

A

IgA vasculitis (henoch-schonlein)

99
Q

Dx this

A
100
Q

Describe the Mononeuritis Multiplex

A
  • ● Mononeuritis multiplex
    • ○ Peripheral neuropathy–asymmetric or symmetric ○ Seen in up to 45% of patients with EGPA (Churg-Strauss)
    • Causes
      • ■ Leprosy (most common)
      • ■ DM
      • ■ Vasculitis
      • ■ Sarcoidosis
      • ■ Amyloidosis
      • ■ Malignancy
      • ■ Neurofibromatosis
      • ■ HIV infection
      • ■ Idiopathic multifocal motor neuropathy
101
Q

How do you DX- Churg-Strauss syndrome?

A
102
Q

In Small vessel vasculiitis- Granulomatosis with polyangiitis (Wegeners), what clinical features do expect to find?

A
103
Q

Things to look for in DX- granulomatosis with polyangiitis (wegeners)

A
104
Q

Describe Secondary Vasculitis: Cryoglobulinemic vasculitis

A
105
Q

What are the important questions to ask yourself before a case?

A
106
Q

Describe Small Vessel Vasculitis

A
  • Cutaneous post-capillary venules- palpable purpura
  • Glomerular capillaries- haematuria, red cell casts in urine, proteinuria, decline in renal function
  • Pulmonary capillaries- lung hemorrhage
107
Q

Describe Secondary Vasculitis

A
108
Q

Describe Primary Vasculitis

A
109
Q

What are the key points of Vasculitis?

A
  • Damage to vessels
  • Every organ system ‘syndrome’ can be involved, or single organ
  • ‘Polyangiitis’ usually denotes small vessel
  • Rare (delays dx)
  • Primary or secondary to another disease
  • Presentation: varies from minimal to life-threatening
115
Q

Describe Large Vessel Vasculitis

A

Takayasu

Giant Cell

116
Q

Describe Large Vessel Vasculitis

A

Takayasu

Giant Cell

119
Q

What are the Clinical features of Small vessel vasculiitis- microscopic polyangiitis

A

Skin, joints, kidneys, lungs, nerves, eyes

Blood tests: pANCA/anti-MPO

120
Q

What are the Clinical features of Small vessel vasculiitis- microscopic polyangiitis

A

Skin, joints, kidneys, lungs, nerves, eyes

Blood tests: pANCA/anti-MPO

124
Q

What does this describe?

Asthma, URT, nerves, gut, heart
Histology: eosinophilic vasculitis
Blood tests: high eosinophils, ANCA+ve in <50%

A

Small vessel vasculiitis- Church-Strauss syndrome
Eosinophilic Granulomatosis with polyangiitis

125
Q

What does this describe?

Asthma, URT, nerves, gut, heart
Histology: eosinophilic vasculitis
Blood tests: high eosinophils, ANCA+ve in <50%

A

Small vessel vasculiitis- Church-Strauss syndrome
Eosinophilic Granulomatosis with polyangiitis

129
Q

What does this describe?

Pulseless disease. Med-Lg vessels, aortic arch and branches

A

Takayasu arteritis

130
Q
  • What does this describe?​
    • Febrile illness in children. Coronary involvement in 20% w/MI
    • Acute fever with: lymphadenopathy, rash, oral/lip erythema
A

Kawasaki disease

131
Q
  • What does this describe?​
    • Febrile illness in children. Coronary involvement in 20% w/MI
    • Acute fever with: lymphadenopathy, rash, oral/lip erythema
A

Kawasaki disease

132
Q

What does this describe?

Pulseless disease. Med-Lg vessels, aortic arch and branches

A

Takayasu arteritis

Cardio All (8 decks)

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