04.08 - Aneurysms, PVD (Nichols) Flashcards
(158 cards)
1
Q
Vasculitis Cause of AA
A
Giant Cell Arteritis
2
Q
1/3 of PAN patients have what infection
A
Chronic Hep B
3
Q
2 Age groups for Aortic Dissection
A
40-60 w antecedent HTN; younger pts with CT disorders
4
Q
2 common features of Microscopic Poly
A
Necrotizing Glomerulonephritis and Pulmonary Capillaritis
5
Q
2 Immune Axes in GCA
A
TH1: IL-12 - IFN-gamma; TH17: IL-1 - IL-23
6
Q
2 most common pathogenic mechanisms of vasculitis
A
Immune-mediated inflammation, Direct Vascular Invasion by Infectious Pathogens
7
Q
2 Most important causes of Aortic Aneurysms
A
Athersclerosis and HTN
8
Q
2nd most commonly affected vessels in Takayasu
A
Pulmonary in 50% of cases
9
Q
3 Major CV Sequelae of Kawasaki if untreated
A
Coronary Artery Aneurysms, MI, SCD
10
Q
4 Top Presentations of Wegener’s
A
Bilateral Pneumoitis, Chronic Sinusitis, Mucosal ulcerations of Nasopharynx, Renal disease
11
Q
A patient with AAA is at significantly increased risk for what other events
A
IHD and Stroke
12
Q
Ab’s in PAN
A
Characteristically has none
13
Q
Ab’s present in Wegener’s
A
PR3-ANCAs
14
Q
Ab’s to Endothelial cells =
A
Kawasaki Disease
15
Q
Abundant Basophilic Debris (Nuclear Dust) + Geographic Pattern Necrosis is called
A
Pathergic Necrosis
16
Q
Acute Arterial Occlusion is often a __ emergency
A
Surgical
17
Q
Acute Arterial Occlusion is usually due to thromboemboli from
A
Heart (mural > LA)
18
Q
Affect of ANCA’s on neutrophils
A
Can directly activate them
19
Q
Age group of Kawasaki
A
Usually self-limited illness of infancy and childhood
20
Q
Age group of PAN
A
Young adults
21
Q
ANCA titers generally mirror
A
Clinical severity
22
Q
Aneurysms of what size are managed surgically
A
5cm or larger
23
Q
Anti-myeloperoxidase =
A
p-ANCA
24
Q
Anti-proteinase 3 =
A
C-ANCA
25
Antibodies usually associated with PAN
None
26
Arteries involved in Giant Cell Arteritis
Temporal and Ophthalmic, Thoracic Aorta
27
Atheroembolization is associated with
Peripheral Blood Eosinophilia
28
Bluish Myxoid Ground Substance =
Young
29
Buerger Disease is aka
Thromboangiitis Obliterans
30
Buerger disease occurs almost exclusively in
heavy tobacco smokers, usually develops before age 35
31
C-ANCA =
Anti-proteinase 3
32
Cause of ischemia in GCA
Nodular intimal thickening and occasional thromboses
33
Classic Clinical Symptom of Aortic Dissection
Sudden onset of excrutiating tearing or stabbing pain, usually beginning in the anterior chest, radiating to the back between the scapulae, and moving downward
34
Clinical course of PAN is typically
episodic (long symptom-free intervals)
35
Clinical Significance of Kawasaki
Involvement of Coronaries
36
Culprit immune cell in GCA
T Cell-mediated response against unknown vessel wall antigen
37
Cystic Medial Degeneration
Fibrosis, Inadequate ECM synthesis, Accumulation of Amorphous Proteoglycans
38
Decreased TIMP expression tips balance toward
Net ECM degradation
39
Demographic of Giant Cell Arteritis
Elderly white females of Northern European descent
40
Distinct type of AAA characterized by dense periaortic fibrosis containing abundant lymphoplasmacytic inflammation with mac's and giant cells
Inflammatory AAA
41
Distinction between GCA and Takayasu is made largely on basis of
patient age (50 is cutoff)
42
Dx of Giant Cell Arteritis is based on
High ESR and + Bx
43
Ectasia vs Aneurysm
Ectasia is not discrete and localized
44
Exception of Vasculitis that also affects veins
G w P (Wegener's)
45
Fever, Fatigue, Weight Loss, Pain in side of head, Ocular Symptoms
GCA
46
Fibrinoid Necrosis is most common in
PAN
47
Fibrosis, Inadequate ECM synthesis, Accumulation of Amorphous Proteoglycans
Cystic Medial Degeneration
48
Findings in Wegener's
Triad: Granulomas, Vasculitis, Glomerulonephritis
49
G w/ P: Granulomatous vasculitis of both arteris and veins in lungs will cause
Areas of pathergic Necrosis --> Nodules --> Central Cavitation
50
G w/ P: Patient will likely have
Granulomatous Vasculitis and Glomerulonephritis
51
GCA is associated with what rheumatic disease
Polymyalgia Rheumatica
52
GCA takes the form of
Chronic, typically granulomatous, inflammation of large to small arteries (mainly head)
53
GCA: increased IFN-g correlates with production of
VEGF and PDGF --> Lumen stenosis
54
GCA: what induces mac's to for multinucleated giant cells
IFN-gamma
55
GCA: what leads to lumen stenosis
IFN-g --> VEGF and PDGF
56
Granulomas, Vasculitis, Glomerulonephritis
Triad of Findings in Wegener's
57
Granulomatosis with Polyangiitis affects which primary 3 parts of body
(1) Lungs; (2) Kidney; (3) Nasopharynx
58
Granulomatous Vasculitis with Eosinophilia
Churg-Strauss
59
heavy tobacco smokers, usually develops before age 35
Buerger disease occurs almost exclusively in
60
IL-12 and IL-18 in GCA upregulate release of what from T cells
IFN-gamma; CCL19 and CCL21
61
In Marfarn, defective synthesis of Fibrillin leads to
abnormal sequestration of TGF-beta
62
In up to 30% of patients with PAN, vasculitis is attributable to
Immune complexes to Hep B
63
In whom is PAN most common
Middle-aged males
64
Inflammation in Aortic Dissection
Characteristically absent
65
Inflammatory Giant Cells indicated
TH1's secreting IFN-gamma
66
Kawasaki Disease is AKA
Mucocutaneous Lymph Node Syndrome
67
Leukocytoclastic Vasculitis is aka
Microscopic Poly
68
Luminal thrombosis with small microabscesses
Buerger Disease
69
Major cause of death in PAN patients
Renal involvement (renal arteries most likely to be affected)
70
Major Determinant of AA Rupture
Diameter
71
Major risk factor for Aortic Dissection
HTN
72
Manifestation of TAA
(1) Encroachment on mediastinal structures; (2) Persistent cough; (3) Pain from bone erosion; (4) Cardiac disease
73
Microscopic Poly is characterized by
Segmental fibrinoid necrosis of media with focal transmural necrotizing lesions
74
Morphology of Kawasaki resembles
PAN
75
Most cases of Microscopic Poly are associated with
MPO-ANCA
76
Most common clinical manifestation of immune complex dep
Skin Lesions
77
Most common form of vasculitis among elderly in developed countries
GCA
78
Most common symptom in GCA
Headache
79
Most frequent histologically detectable lesion in Aortic Dissection
Cystic Medial Degeneration
80
Most lesions in Microscopic Poly are
Pauci-Immune
81
MPO-ANCA is fka
P-ANCA
82
MPO-ANCA's (fka P-ANCA) are associated with
Microscopic Polyangiitis, Churg-Straus
83
Multifocal necrotizing granulomatous vasculitis with a surrounding fibroblastic proliferation
Wegener's
84
Mutations in TGF-beta or down-stream signaling pathways result in
Defective Elastin and Collagen synthesis --> Aneurysms
85
Mycotic AAA's occur when
Circulating microorganisms seed the aneurysm wall or associated thrombus
86
Necrosis seen in PAN
Fibrinoid
87
Necrotizing Glomerulonephritis and Pulmonary Capillaritis
2 common features of Microscopic Poly
88
Necrotizing vasculitis that generally affects capillaries, as well as small arterioles and venules
Microscopic Polyangiitis
89
Ocular disturbances and marked weakening of pulses in upper extremities
Takayasu Arteritis is characterized principally by
90
Other name of Kawasaki
Mucocutaneous Lymph Node Syndrome
91
p-ANCA =
Anti-myeloperoxidase
92
Pathergic Necrosis
Abundant Basophilic Debris (Nuclear Dust) + Geographic Pattern Necrosis is called
93
Pathogenesis of AA involves increased
Matrix Metalloproteinases
94
Pathology of PAN
Segmental transmural necrotizing vasculitis of Small, Medium Arteries with Fibrinoid Necrosis (and lesions at diff stages)
95
Pauci-immune =
Vascular lesions do not typically contain demonstrable Ab and Complement
96
PR3-ANCA (fka C-ANCA) is associated with
Wegener Granulomatosis
97
PR3-ANCA fka
C-ANCA
98
Primary associated disease with C-ANCA
Granulomatosis with Polyangiitis (Wegener's)
99
Primary associated disease with P-ANCA
Microscopic Polyangiitis
100
PVD: Pain at rest, ulcers =
Severe disease
101
Rising ANCA titers usually predict
Disease Relapse
102
Segment fibrinoid necrosis of the media with focal transmural necrotizing lesions
Microscopic Poly
103
Segmental transmural necrotizing inflammation of small to medium-sized arteries
PAN
104
Segmental transmural necrotizing vasculitis of Small, Medium Arteries with Fibrinoid Necrosis (and lesions at diff stages)
Pathology of PAN
105
Sharply segmental acute and chronic transmural vasculitis of medium and small arteries
Buerger Disease (thromboangiitis obliterans)
106
Signature cytokine of Th1 cells
IFN-gamma
107
Signs and Symptoms of Acute Arterial Occlusion
Pain, Pallow, Paralysis, Paresthesia, Pulselessness (Poikylothermia)
108
Steroid tx cannot affect expansion of which population in GCA
TH1 cells
109
Striking infiltration of vessels and perivascular tissues by eosinophils
Churg-Strauss
110
Sudden onset of excrutiating tearing or stabbing pain, usually beginning in the anterior chest, radiating to the back between the scapulae, and moving downward
Classic Clinical Symptom of Aortic Dissection
111
Systemic manifestation of Giant Cell Arteritis
Fever
112
Systemic manifestations of GCA are most responsive to
Reduction of TH17 but prednisone
113
T/F: All cases of GCA have granulomas and giant cells
False, 25% do not
114
T/F: ANCA autoantibodies make up most of the immune complex deposition
FALSE
115
T/F: In PAN, all lesions are at same level of progression
False, characteristically all stages of activity coexist, indicating ongoing and recurrent pathogenic insults
116
T/F: Kawasaki disease is usually fatal
False, usually self-limited of infancy and chilhood
117
T/F: Recognizable medial damage is prereq for Aortic Dissection
FALSE
118
T/F: Takayasu has giant cells
TRUE
119
TAA's are most commonly associated with
HTN and Marfan Syndrome
120
Takayasu Arteritis classically affects
Aortic Arch and Arch Vessels
121
Takayasu Arteritis is characterized principally by
Ocular disturbances and marked weakening of pulses in upper extremities
122
Takayasu Arteritis manifests with
Transmural scarring and thickening of the aorta - with severe luminal narrowing of major branch vessels
123
Three Steps of Mycotic Aneurysms
(1) Embolization of septic embolus; (2) Extension of an adjacent suppurative process; (3) Direct infection of aterial wall
124
Triad of Findings in Wegener's
Granulomas, Vasculitis, Glomerulonephritis
125
Tx of Giant Cell Arteritis
Steroids
126
Tx of Kawasaki
IV IG and Aspirin
127
Tx of Wegener's
Cyclophosphamide, Corticosteroids
128
Type B Aneurysm means
Below Aortic Arch
129
Typical Wegener's patient
40 year old male
130
Unlike in PAN, all lesions in Microscopic Poly
tend to be of same age
131
Upper Respiratory Tract Ulcerations =
Granulomatosis with Polyangiitis
132
Vascular lesions of Churg-Strauss differ from PAN and Micro Poly by presence of
Granulomas, Eosinophils
133
Vasculitis w/ Eosinophilia, Asthma, and Granulomas
Churg-Straus
134
Vasculitis w/ Granulomas but no Asthma
Wegener's
135
Vasculitis w/out Asthma or Granulomas
Microscopic Polyangiitis
136
Vessels affected in Microscopic Poly
Capillaries and small vessels
137
What causes Cystic Medial Degeneration
Luminal narrowing of aortic vasa vasorum leading to ischemia of outer media
138
What initiates the immune response in Giant Cell Arteritis
Dendritic Cells
139
What is a rare cause of Obliterative Endarteritis of Aortic Vasa Vasorum
Tertiary Syphillis
140
What is absent in Microscopic Poly
Granulomatous inflammation
141
What is attacked in Giant Cell Arteritis
Calcified Int Elastic Lamina in arteries (Calcified Atrophic T Media of Thoracic Aorta)
142
What is characterized by focal acute and chronic inflammation of medium-sized and small arteries, especially tibial and radial, associated with thrombosis
Buerger Disease
143
What is likely causing endothelial cell injury in vasculitis
ANCA-activated neutrophils
144
What is produced and released by Dendritic Cells in GCA
IL-12 and IL-18
145
What molecules are mediators of matrix degeneration, intimal hyperplasia, and luminal narrowing in GCA
MMP-2 and -9
146
What regulates SM cell proliferation and matrix synthesis
TGF-beta
147
What size vessels do GCA affect
Large
148
What size vessels does Churg-Straus affect
Small
149
What size vessels does Kawasaki affect
Medium
150
What size vessels does PAN affect
Medium
151
What size vessels does Takayasu affect
Large
152
What size vessels does Wegener's affect
Small
153
What vessels does PAN commonly affect
Medium Muscular: Renal and Visceral (spares pulmonary)
154
Which cells are circulating systemically in GCA
TH17
155
Which has better prognosis: Type A or B Aortic Dissection?
Type B
156
Which Type of Aortic Aneurysm can be managed more conservatively
Type B
157
Why is Giant Cell Arteritis almost exclusively in the eldery
Prereq of Arterial Calcification
158
With progression of Takayasu, ___ signs and symptoms dominate clinical picture
Vascular: Reduced UE BP/Pulse, Neurologic, Ocular disturbances
