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MSK/Derm > SM 249: Vasculitis > Flashcards

SM 249: Vasculitis Flashcards

1
Q

What are the large vessels?

A

Aorta and it’s branches: elastic arteries

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2
Q

What are elastic arteries?

A

Arteries that allow for expansion and recoil, maintaining blood pressure during Diastole

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3
Q

Are large vessels elastic or muscular arteries?

A

Large vessels like the Aorta and it’s branches are elastic arteries

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4
Q

What are muscular arteries?

A

Arteries with more smooth muscle and some autonomic control as a result

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5
Q

What are Arterioles?

A

Arteries with the most smooth muscle and the most autonomic control, allowing them to control local blood flow

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6
Q

Describe the structure of a capillary?

A

A single endothelial layer optimized for nutrient and gas exchange

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7
Q

How does vascular inflammation effect vessels?

A

Vascular inflammation can lead to obstruction, loss of vessel integrity, and aneurysm formation

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8
Q

How are Vasculitis classified?

A

By vessel size:

Large Vessel Vasculitis

Medium Vessel Vasculitis

ANCA Small Vessel Vasculitis

Immune Complex Small Vessel Vasculitis

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9
Q

What are the large vessel vasculitis?

A

Takayasu Arteritis and Giant Cell Arteritis

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10
Q

What are the medium vessel vasculitis?

A

Polyarteritis Nodosa

Kawasaki Disease

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11
Q

What are the ANCA associated small vessel vasculitis?

A

MPA
GPA
EPA

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12
Q

What are the Immune Complex small vessel vasculitis?

A

Cryoglobulinemic Vasculitis
IgA Vasculitis
Hypocomplemntemic Utricarial Vasculitis
Anti-GBM Disease

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13
Q

What is the most common form of arteritis?

A

Giant Cell Arteritis

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14
Q

Who does Giant Cell Arteritis effect the most?

A

Scandinavian women more often than men, around 73 y/o

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15
Q

What causes Giant Cell Arteritis?

A

Uknown

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16
Q

What vessels are effected by Giant Cell Arteritis?

A

Multinucleated giant cells and granulomas in the Internal Elastic Lamina of large vessels

Cranial involvement

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17
Q

How does Giant Cell Arteritis present?

A

New headache, polymyalgia, jaw claudication, vision loss, and constitutional symptoms

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18
Q

What lab abnormalities accompany GCA?

A

Elevated ESR and CRP as well as Thrombocytosis

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19
Q

What imaging should be ordered if GCA is suspected?

A

Temporary Artery Ultrasound and MRA

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20
Q

What artery should be biopsied and how quickly in GCA?

A

Biopsy the Temporal artery ASAP

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21
Q

How is GCA treated?

A

High dose corticosteroids and Tocilizumab

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22
Q

What is Tocilizumab?

A

An anti-IL-6 therapy

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23
Q

What is Takayasu Arteritis?

A

A large vessel vasculitis that can affect any artery but mainly the Aorta/Sublcavian/Carotids, leading to stenosis, dilation, and aneurysm formation

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24
Q

Who is affected by Takayusu Arteritis?

A

Most common in Asia, women > men and age < 40

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25
How does Takayusu Arteritis present?
Extremity pain/claudication, bruits, lightheadedness, and diminished pulses or large BP difference
26
What lab abnormalities is Takayusu Arteritis associated with?
No specific abnormalities, ESR/CRP may or may not be elevated
27
What pathology is associated with Takayusu Arteritis?
Granulomatous panarteritis, intimal hyperplasia, and fixed stenosis/occlusions
28
What imaging should be ordered with Takayusu Arteritis?
CTA/MRA > Angiography
29
What is the treatment for Takayusu Arteritis?
High dose corticosteroids (empirically)
30
Why is early recognition and initiation of therapy critical Takayusu Arteritis?
Aortic regurg and acute MI are frequent complications that lead to death
31
What is Polyarteritis Nodosa?
A systemic necrotizign vasculitis of medium-sized muscular arteries
32
What causes Polyarteritis Nodosa?
Associated with HBV but may also be idiopathic
33
Who is effected by Polyarteritis Nodosa?
Men between the age of 40-60 typically
34
What is the pathology of Polyarteritis Nodosa?
Transmural inflammation without giant cells or granulomas
35
How does PAN present?
Cutaneous nodules, Renal Disease, Neuropathy, GI Ischemia
36
What is the cause of secondary PAN?
Typically Hepatitis B Virus
37
What is the treatment of PAN?
Glucocorticosteroids and Methotrexate/Azathioprine/Cyclophosphamide Test for and treat HBV as well if needed
38
What is Kawasaki disease?
A self-limited febrile vasculitis of medium-sized muscular and coronary arteries in young children
39
Who does Kawasaki Disease effect?
Boys > girls, Asian, Less than 5 years old
40
What is the pathology of Kawasaki Disease?
Destruction of luminal endothelial cells, elastic lamina
41
What are the laboratory findings associated with Kawasaki Disease?
Left-shift Leukocytosis (more immature cells) Thrombocytosis Transaminitis
42
What is the treatment for Kawasaki Disease?
High Dose Aspirin for the fever as well as IVIG to reduce coronary artery aneurysms
43
What is the role of Immunofluorescence in ANCA vasculitis?
Immunofluorescence identifies an ANCA pattern as either cytoplasmic or perinuclear
44
What is the role of ELISA in ANCA vasculitis?
Identifies the antigen: MPO = p-ANCA and PR3 = c-ANCA
45
What is GPA?
A small vessel vasculitis associated with c-ANCA/PR3
46
Who does GPA effect?
Average onset at 40-50 years old in Caucasians
47
What is the pathology of GPA?
Necrotizing granulomas without immune deposits
48
How does GPA present?
Pulmonary and airway disease as well as venous thrombosis and Glomerulonephritis
49
What is MPA?
A small vessel vasculitis
50
Who gets MPA?
Middle aged people
51
What is the pathology of MPA?
Necrotiing arteritis without granulomas or immune deposits
52
What areas are normally effected by MPA?
Kidneys = Glomerulonephritis | Pulmononary capillaries
53
What is the treatment for MPA and GPA?
Steroids, Ritixumiab
54
What is EGPA?
Eosinphilic necrotizing vasculitis that often lacks ANCA markers
55
Who gets EGPA?
Men > Women, often occurs between 25 - 45
56
What Ig is elevated in EGPA?
IgE = adult onset asthma
57
What is the patholog of EGPA?
Necrotizing vasculitis with Eosinophilic infiltration and granulomas
58
How does EGPA present?
Asthma, allergic rhinitis into Eosinophilic inflammation and finally Vasculitic phase
59
What happens in the Eosinophilic phase of EGPA?
Tissue and periphary invasion by Eosinophils
60
What occurs during the Vasculitic phase of EGPA?
Cutaneous, cardiac, and reneal manifestations of EGPA
61
How should EGPA be treated?
Glucocorticosteroids, Cyclophosphamide, Mepolizumab
62
What is Mepolizumab?
An anti IL-5 drug for treating EGPA
63
Which vasculitis causes skun purpursa?
MPA
64
Which vasculitis involves the peripheral and central nervous systems?
EGPA
65
Which vasculitis causes upper airway disease?
GPA
66
What is anti-GBM disease?
An immune complex small vessel vasculitis that follows URI
67
Who gets anti-GBM disease?
Older children and adults typcially after a URI
68
How does anti-GBM disease present?
Acute renal failure + alveolar hemorrhage
69
What antibodies circulate in anti-GBM disease?
Anti-GBM antibodies, which are used to diagnose anti-GBM disease
70
What is the treatment for anti-GBM disease?
Plasmapheresis and glucocorticoids + cyclophosphamide
71
What are Cryoglobulins?
Ig's or a mixture of Ig's and Complement that precipitate at temperatures less than 37C
72
What are the 3 types of Cryoglobullinemia?
Type I = Monoclonal Ig Type II = Mixed Monoclonal Ig and Polyclonal Ig Type III = Polyclonal IgG and IgM
73
What condition is associated with Cryoglobulinemia?
Hepatitis C
74
How does cryoglobulinemia effect the blood?
Hyperviscosity leads to digitial ischemia and ski necrosis
75
What is IgA vasculitis?
A disease of children primary that occurs after a URI
76
What is the pathology of IgA vasculitis?
IgA and C3 complexes in a blood vessel with granulocytes in the walls of vessels
77
How does IgA vasculitis present?
In a child: purpura rash, abdominal pain, myalgias and hematuria
78
How is IgA vasculitis?
Resolves on it's own, but add Glucocorticoids to prevent nephritic disease
79
What is Behcets syndrome?
A mixed vessel vasculitis found in the Miditerranean, Middle and Far East
80
How does Behcets syndrome present?
Oral ulcers, Genital ulcers, and ocular changes
81
How is Behcets treated?
Organ-based treatment: colchicine, glucocorticoids, TNF inhibitors
82
What constitutional symptoms accompany all vasculitis?
Fevers, weight loss, fatigue
83
Which vasculitis effect the elderly?
GCA, ANCA
84
Which vasculitis effect children?
Kawasaki, Takayasus, IgA Vasculitis
85
Which vasculitis effect the kiddney?
ANCA, Cryo, IgA Vasculitis
86
Which vasculitis effect the abdomen?
IgA Vasculitis
87
Which vasculitis presents as an early asthma/allergy reaction?
EGPA
88
What is the point of a biopsy in vasculitis?
Confirm a diagnosis, preferably before starting therapy

MSK/Derm (46 decks)

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