SM 249: Vasculitis Flashcards

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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
Q

How does Takayusu Arteritis present?

A

Extremity pain/claudication, bruits, lightheadedness, and diminished pulses or large BP difference

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26
Q

What lab abnormalities is Takayusu Arteritis associated with?

A

No specific abnormalities, ESR/CRP may or may not be elevated

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27
Q

What pathology is associated with Takayusu Arteritis?

A

Granulomatous panarteritis, intimal hyperplasia, and fixed stenosis/occlusions

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28
Q

What imaging should be ordered with Takayusu Arteritis?

A

CTA/MRA > Angiography

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29
Q

What is the treatment for Takayusu Arteritis?

A

High dose corticosteroids (empirically)

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30
Q

Why is early recognition and initiation of therapy critical Takayusu Arteritis?

A

Aortic regurg and acute MI are frequent complications that lead to death

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31
Q

What is Polyarteritis Nodosa?

A

A systemic necrotizign vasculitis of medium-sized muscular arteries

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32
Q

What causes Polyarteritis Nodosa?

A

Associated with HBV but may also be idiopathic

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33
Q

Who is effected by Polyarteritis Nodosa?

A

Men between the age of 40-60 typically

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34
Q

What is the pathology of Polyarteritis Nodosa?

A

Transmural inflammation without giant cells or granulomas

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35
Q

How does PAN present?

A

Cutaneous nodules, Renal Disease, Neuropathy, GI Ischemia

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36
Q

What is the cause of secondary PAN?

A

Typically Hepatitis B Virus

37
Q

What is the treatment of PAN?

A

Glucocorticosteroids and Methotrexate/Azathioprine/Cyclophosphamide

Test for and treat HBV as well if needed

38
Q

What is Kawasaki disease?

A

A self-limited febrile vasculitis of medium-sized muscular and coronary arteries in young children

39
Q

Who does Kawasaki Disease effect?

A

Boys > girls, Asian, Less than 5 years old

40
Q

What is the pathology of Kawasaki Disease?

A

Destruction of luminal endothelial cells, elastic lamina

41
Q

What are the laboratory findings associated with Kawasaki Disease?

A

Left-shift Leukocytosis (more immature cells)
Thrombocytosis
Transaminitis

42
Q

What is the treatment for Kawasaki Disease?

A

High Dose Aspirin for the fever as well as IVIG to reduce coronary artery aneurysms

43
Q

What is the role of Immunofluorescence in ANCA vasculitis?

A

Immunofluorescence identifies an ANCA pattern as either cytoplasmic or perinuclear

44
Q

What is the role of ELISA in ANCA vasculitis?

A

Identifies the antigen: MPO = p-ANCA and PR3 = c-ANCA

45
Q

What is GPA?

A

A small vessel vasculitis associated with c-ANCA/PR3

46
Q

Who does GPA effect?

A

Average onset at 40-50 years old in Caucasians

47
Q

What is the pathology of GPA?

A

Necrotizing granulomas without immune deposits

48
Q

How does GPA present?

A

Pulmonary and airway disease as well as venous thrombosis and Glomerulonephritis

49
Q

What is MPA?

A

A small vessel vasculitis

50
Q

Who gets MPA?

A

Middle aged people

51
Q

What is the pathology of MPA?

A

Necrotiing arteritis without granulomas or immune deposits

52
Q

What areas are normally effected by MPA?

A

Kidneys = Glomerulonephritis

Pulmononary capillaries

53
Q

What is the treatment for MPA and GPA?

A

Steroids, Ritixumiab

54
Q

What is EGPA?

A

Eosinphilic necrotizing vasculitis that often lacks ANCA markers

55
Q

Who gets EGPA?

A

Men > Women, often occurs between 25 - 45

56
Q

What Ig is elevated in EGPA?

A

IgE = adult onset asthma

57
Q

What is the patholog of EGPA?

A

Necrotizing vasculitis with Eosinophilic infiltration and granulomas

58
Q

How does EGPA present?

A

Asthma, allergic rhinitis into Eosinophilic inflammation and finally Vasculitic phase

59
Q

What happens in the Eosinophilic phase of EGPA?

A

Tissue and periphary invasion by Eosinophils

60
Q

What occurs during the Vasculitic phase of EGPA?

A

Cutaneous, cardiac, and reneal manifestations of EGPA

61
Q

How should EGPA be treated?

A

Glucocorticosteroids, Cyclophosphamide, Mepolizumab

62
Q

What is Mepolizumab?

A

An anti IL-5 drug for treating EGPA

63
Q

Which vasculitis causes skun purpursa?

A

MPA

64
Q

Which vasculitis involves the peripheral and central nervous systems?

A

EGPA

65
Q

Which vasculitis causes upper airway disease?

A

GPA

66
Q

What is anti-GBM disease?

A

An immune complex small vessel vasculitis that follows URI

67
Q

Who gets anti-GBM disease?

A

Older children and adults typcially after a URI

68
Q

How does anti-GBM disease present?

A

Acute renal failure + alveolar hemorrhage

69
Q

What antibodies circulate in anti-GBM disease?

A

Anti-GBM antibodies, which are used to diagnose anti-GBM disease

70
Q

What is the treatment for anti-GBM disease?

A

Plasmapheresis and glucocorticoids + cyclophosphamide

71
Q

What are Cryoglobulins?

A

Ig’s or a mixture of Ig’s and Complement that precipitate at temperatures less than 37C

72
Q

What are the 3 types of Cryoglobullinemia?

A

Type I = Monoclonal Ig

Type II = Mixed Monoclonal Ig and Polyclonal Ig

Type III = Polyclonal IgG and IgM

73
Q

What condition is associated with Cryoglobulinemia?

A

Hepatitis C

74
Q

How does cryoglobulinemia effect the blood?

A

Hyperviscosity leads to digitial ischemia and ski necrosis

75
Q

What is IgA vasculitis?

A

A disease of children primary that occurs after a URI

76
Q

What is the pathology of IgA vasculitis?

A

IgA and C3 complexes in a blood vessel with granulocytes in the walls of vessels

77
Q

How does IgA vasculitis present?

A

In a child: purpura rash, abdominal pain, myalgias and hematuria

78
Q

How is IgA vasculitis?

A

Resolves on it’s own, but add Glucocorticoids to prevent nephritic disease

79
Q

What is Behcets syndrome?

A

A mixed vessel vasculitis found in the Miditerranean, Middle and Far East

80
Q

How does Behcets syndrome present?

A

Oral ulcers, Genital ulcers, and ocular changes

81
Q

How is Behcets treated?

A

Organ-based treatment: colchicine, glucocorticoids, TNF inhibitors

82
Q

What constitutional symptoms accompany all vasculitis?

A

Fevers, weight loss, fatigue

83
Q

Which vasculitis effect the elderly?

A

GCA, ANCA

84
Q

Which vasculitis effect children?

A

Kawasaki, Takayasus, IgA Vasculitis

85
Q

Which vasculitis effect the kiddney?

A

ANCA, Cryo, IgA Vasculitis

86
Q

Which vasculitis effect the abdomen?

A

IgA Vasculitis

87
Q

Which vasculitis presents as an early asthma/allergy reaction?

A

EGPA

88
Q

What is the point of a biopsy in vasculitis?

A

Confirm a diagnosis, preferably before starting therapy