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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
What ANCA is associated with Churg-Strauss syndrome?
MPO-ANCA
28
Thromboangiitis Obliterans
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What term is described: A small irculating antibody that reacts with neutrophil cytoplasmic antigens, common in many patients with Vasculitis
Anti-Neutrophil Cytoplasmic Antibodies (ANCAs)
30
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).
Polyarteritis nodosa
32
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?
Giant Cell Arteritis Steroids or Anti-TNF therapies
33
Which ANCA is a 'Neutrophil Azurophilic Granule Constituent'?
Anti-proteinase-3 PR3-ANCA
34
What is the described condition... Chronic inflammatory disorder, typically with granulomatous inflammation that principally affects Large to small size arteries in the head?
Giant cell (temporal) arteritis
35
What is the 1st line treatment for Kawasaki Disease?
IVIG & aspirin
36
What is Tx of Microscopic Polyangiitis (2)?
Immunosuppressive and removal of offending agent.
37
What vasculitis that we learned about often leads to cardiomyopathy?
Churg-Strauss Syndrome
38
What is important about the diagnosis of Giant cell arteritis ?
This disorder can cause sudden blindness, so hurry it up
39
Giant Cell Arteritis
40
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.
Polyarteritis Nodosa
41
What vasculitis disorder do you think of when you think of heavy tobacco smokers under 35?
Thromboangiitis Obliterans.
42
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.
Microscopic polyangiitis
44
If you find Vasculitis strictly in the LARGE vessels, what conditions are you thinking?
Granulomatous diseases (Giant Cell Arteritis and Takayuki Arteritis)
45
What is the most common form of Vasculitis among older adults in developed countries?
Giant Cell Arteritis
46
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/
Giant Cell Arteritis
47
What is the described condition... Clinical Presentation: Hemoptysis, Hematuria, Proteinuria, Abdominal Pain, Muscle plain and palpable cutaneous purpura.
Microscopic polyangiitis
48
Describe how Autoreactive T-Cells cause Vasculitis.
They cause injury though the formation of granulomas
49
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.
Kawasaki Disease
50
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
Churg-Strauss Syndrome
51
What are the two most common/important ANCAs? What are they typically associated with?
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
Where does takayasu arteritis mainly affect?
Aortic arch and arch vessels (pulmonary arteries are involved in 50% of pts)
53
What is the 'triad' of Granulomatosis with Polyangiitis?
54
What ANCA is associated with granulomatosis with polyangiitis?
Anti-Proteinase-3 (PR3-ANCA)
55
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.
T T
56
What is the described condition... Presents like Giant Cell Arteritis but pt is under 50.
Takayasu arteritis
57
Untreated, Polyarteritis Nodosa is typically fatal, what organ(s) does it affect mainly to inflict death?
Kidneys
58
What are the two organs you think of when you think of Microscopic Polyangiitis?
Lungs and kidneys.
59
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.
Polyarteritis nodosa
60
What ANCA is a lysosomal granule constituent involved in oxygen free radical generation?
MPO-ANCA
61
Takayasu Arteritis
62
What is important to remember about the morphology of Giant Cell Arteritis?
It is very patchy. The involved arterial segments exhibit NODULAR INTIMAL THICKENING that can reduce vessel diameter and cause distal ischemia.
63
Is systemic lupus erythematous a small vessel Vasculitis or a large vessel Vasculitis?
Small
64
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.
Thromboangiitis Obliterans
65
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.
Takayasu Arteritis
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Polyarteritis Nodosa
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What ANCA is associated with microscopic polyangiitis?
MPO-ANCA
69
Giant Cell Arteritis
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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.
Kawasaki disease
71
What is the described condition... Necrotizing Vasculitis that generally affects capillaries as well as small ARTERIOLES and venules.
Microscopic Polyangiitis
72
What is an example of an Anti-Endothelial Cell antibody caused disorder?
Kawasaki
74
(T/F): Giant Cell arteritis can lead to sudden an permanent blindness.
True
75
How do you reach a diagnosis of Giant Cell Arteritis?
Biopsy of temporal arteries
76
What are the key points of Vasculitis?
* 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
Describe Primary Vasculitis
78
Describe Secondary Vasculitis
79
Describe Small Vessel Vasculitis
* Cutaneous post-capillary venules- palpable purpura * Glomerular capillaries- haematuria, red cell casts in urine, proteinuria, decline in renal function * Pulmonary capillaries- lung hemorrhage
80
Describe Medium Vessel Vasculitis
Polyarteritis Nodosa Kawasaki
81
Describe Medium Vessel Vasculitis
Polyarteritis Nodosa Kawasaki
82
What are the important questions to ask yourself before a case?
83
Describe Secondary Vasculitis: Cryoglobulinemic vasculitis
84
Things to look for in DX of microscopic polyangiitis
* 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
Things to look for in DX of microscopic polyangiitis
* 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
Things to look for in DX- granulomatosis with polyangiitis (wegeners)
87
In Small vessel vasculiitis- Granulomatosis with polyangiitis (Wegeners), what clinical features do expect to find?
88
How do you DX- Churg-Strauss syndrome?
89
What are the clinical features of Churg-Strauss syndrome?
90
What are the clinical features of Churg-Strauss syndrome?
91
Describe the Mononeuritis Multiplex
* ● 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
Dx this
93
Dx this
94
Dx this
95
Dx this
96
Dx this
97
What does this describe?​
IgA vasculitis (henoch-schonlein)
98
What does this describe?​
IgA vasculitis (henoch-schonlein)
99
Dx this
100
Describe the Mononeuritis Multiplex
* ● 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
How do you DX- Churg-Strauss syndrome?
102
In Small vessel vasculiitis- Granulomatosis with polyangiitis (Wegeners), what clinical features do expect to find?
103
Things to look for in DX- granulomatosis with polyangiitis (wegeners)
104
Describe Secondary Vasculitis: Cryoglobulinemic vasculitis
105
What are the important questions to ask yourself before a case?
106
Describe Small Vessel Vasculitis
* Cutaneous post-capillary venules- palpable purpura * Glomerular capillaries- haematuria, red cell casts in urine, proteinuria, decline in renal function * Pulmonary capillaries- lung hemorrhage
107
Describe Secondary Vasculitis
108
Describe Primary Vasculitis
109
What are the key points of Vasculitis?
* 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
Describe Large Vessel Vasculitis
Takayasu Giant Cell
116
Describe Large Vessel Vasculitis
Takayasu Giant Cell
119
What are the Clinical features of Small vessel vasculiitis- microscopic polyangiitis
Skin, joints, kidneys, lungs, nerves, eyes Blood tests: pANCA/anti-MPO
120
What are the Clinical features of Small vessel vasculiitis- microscopic polyangiitis
Skin, joints, kidneys, lungs, nerves, eyes Blood tests: pANCA/anti-MPO
124
What does this describe? Asthma, URT, nerves, gut, heart Histology: eosinophilic vasculitis Blood tests: high eosinophils, ANCA+ve in \<50%
Small vessel vasculiitis- Church-Strauss syndrome Eosinophilic Granulomatosis with polyangiitis
125
What does this describe? Asthma, URT, nerves, gut, heart Histology: eosinophilic vasculitis Blood tests: high eosinophils, ANCA+ve in \<50%
Small vessel vasculiitis- Church-Strauss syndrome Eosinophilic Granulomatosis with polyangiitis
129
What does this describe? Pulseless disease. Med-Lg vessels, aortic arch and branches
Takayasu arteritis
130
* What does this describe?​ * Febrile illness in children. Coronary involvement in 20% w/MI * Acute fever with: lymphadenopathy, rash, oral/lip erythema
Kawasaki disease
131
* What does this describe?​ * Febrile illness in children. Coronary involvement in 20% w/MI * Acute fever with: lymphadenopathy, rash, oral/lip erythema
Kawasaki disease
132
What does this describe? Pulseless disease. Med-Lg vessels, aortic arch and branches
Takayasu arteritis

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