Vascular Pathology Flashcards
(129 cards)
1
Q
What factors are related to risk of rupture of berry aneurysms?
A
Size
Episodes of HTN
2
Q
What is the main site for a berry aneurysm?
A
AComm: 40%
3
Q
What is the main site for a berry aneurysm?
A
AComm: 40%
4
Q
What are Charcot-Bouchard aneurysms?
A
Aneurysms occurring in straight segments of small intracerebral arteries in basal ganglia, caused by chronic HTN
5
Q
What is vasculitis?
A
Acute or chronic inflammation that results in structural damage to the vessel wall
6
Q
What is vasculitis?
A
Acute or chronic inflammation that results in structural damage to the vessel wall
7
Q
List 4 types of immune-mediated vasculitis
A
Henoch-Schonlein purpura
Cryoglobulinaemic vasculitis
Serum sickness
Connective tissue diseases
8
Q
Direct Ab attack
A
Goodpastures’ syndrome
Kawasaki disease
9
Q
ANCA associated
A
??
10
Q
Unknown
A
Giant cell (temporal) arteritis ??
11
Q
Unknown
A
Giant cell (temporal) arteritis ??
12
Q
Unknown
A
Giant cell (temporal) arteritis ??
13
Q
How can you tell if there are small vessel changes?
A
Retina Kidney Neuropathy Purpuric rash that won't blanch, usually lower limbs more than upper limbs GI bleeding and infarcts Pancreatitis
14
Q
How can you tell if there are small vessel changes?
A
Retina Kidney Neuropathy Purpuric rash that won't blanch, usually lower limbs more than upper limbs GI bleeding and infarcts Pancreatitis
15
Q
Wegener’s
A
Respiratory tract and kidneys
16
Q
Churg-Strauss
A
Granulomas
Eosinophils
17
Q
Histological changes in small vessel vasculitis
A
May be thrombus Wall often dead with fibrinoid necrosis Inflammation around vessel Blood leaking out of vessel May do other tests to assess whether there are e.g. Igs depositing in cell wall Leukocytoclasis
18
Q
Histological changes in small vessel vasculitis
A
May be thrombus Wall often dead with fibrinoid necrosis Inflammation around vessel Blood leaking out of vessel May do other tests to assess whether there are e.g. Igs depositing in cell wall Leukocytoclasis
19
Q
Pathological process in small vessel vasculitis
A
Infiltrate of neutrophils causing vessel injury
Fibrin deposition (in lumen or vessel wall)
Leakage of RBCs
Lymphocytes and macrophages attract later to help in vessel repair
Fibrois of vessel wall
May progress direct to giant cell inflammation in giant cell vasculitis
20
Q
List 7 small vessel vasculitides
A
Microscopic polyangitis
Wegener’s granulomatosis
Churg-Strauss syndrome
Henoch-Schonlein purpura (IgA deposition)
Cryoglobulinaemic vasculitis (test for cryoglobulins)
Serum sickness
Rheumatoid and lupus vasculitis (Rh factor, anti-dsDNA)
21
Q
List 3 manifestations of small vessel vasculitis
A
Nephritis: usually a necrotising and crescentic GN
Pulmonary haemorrhage: haemorrhagic alveolar capillaritis, heals as interstitial fibrosis
Pulmonary-renal syndrome: in microscopic polyangitis and Goodpastures’ syndrome
22
Q
Goodpasture’s disease
A
Anti-BM Abs
23
Q
List 3 manifestations of small vessel vasculitis
A
Nephritis: usually a necrotising and crescentic GN
Pulmonary haemorrhage: haemorrhagic alveolar capillaritis, heals as interstitial fibrosis
Pulmonary-renal syndrome: in microscopic polyangitis and Goodpastures’ syndrome
24
Q
Goodpastures’
A
Anti-BM Abs
25
List 3 manifestations of small vessel vasculitis
Nephritis: usually a necrotising and crescentic GN
Pulmonary haemorrhage: haemorrhagic alveolar capillaritis, heals as interstitial fibrosis
Pulmonary-renal syndrome: in microscopic polyangitis and Goodpastures' syndrome
Skin: purpura
Abdominal pain: bowel necrosis, ulceration and haemorrhage, ??
26
List 3 manifestations of small vessel vasculitis
Nephritis: usually a necrotising and crescentic GN
Pulmonary haemorrhage: haemorrhagic alveolar capillaritis, heals as interstitial fibrosis
Pulmonary-renal syndrome: in microscopic polyangitis and Goodpastures' syndrome
Skin: purpura (non-blanching, more common in lower limbs, rapid onset)
Abdominal pain: bowel necrosis, ulceration and haemorrhage, ??
27
List 3 manifestations of small vessel vasculitis
Nephritis: usually a necrotising and crescentic GN
Pulmonary haemorrhage: haemorrhagic alveolar capillaritis, heals as interstitial fibrosis
Pulmonary-renal syndrome: in microscopic polyangitis and Goodpastures' syndrome
Skin: purpura (non-blanching, more common in lower limbs)
Abdominal pain: bowel necrosis, ulceration and haemorrhage, ??
28
List 3 manifestations of small vessel vasculitis
Nephritis: usually a necrotising and crescentic GN
Pulmonary haemorrhage: haemorrhagic alveolar capillaritis, heals as interstitial fibrosis
Pulmonary-renal syndrome: in microscopic polyangitis and Goodpastures' syndrome
Skin: purpura (non-blanching, more common in lower limbs)
Abdominal pain: bowel necrosis, ulceration and haemorrhage, ??
Peripheral neuropathy, myalgia, arthralgia: involvement of small arteries and arterioles, may have elevated muscle enzymes
29
Goodpastures'
Anti-BM Abs
30
Pauci-immune vaculitis
??
31
Microscopic polyangitis
Necrotising systemic vasculitis affecting arterioles, capillaries and venules (and sometimes small and medium sized arteries)
Characterised by ??
32
Incidence of MP
1 in 1000
33
MP demographics
Slight M>F predominance
| Mean age 50 years
34
Microscopic polyangitis
Necrotising systemic vasculitis affecting arterioles, capillaries and venules (and sometimes small and medium sized arteries)
Characterised by ??
Segmental vascular necrosis
Infiltrate of neutrophils and monocytes
May have leukocytoclasis - split neutrophils?, fibrin and haemorrhage
Thrombosis in small arteries causing infarction
Healing with fibrosis
Pseudoaneurysms may occur in medium sized arties)
35
MP demographics
Slight M>F predominance
| Mean age 50 years
36
P-ANCA
MP
37
C-ANCA
??
38
C-ANCA
??
39
ANCA-positive
Probably pauci-immune?
40
ANCA-positive
Probably pauci-immune?
41
Wegener's granulomatosis
Granulomatous inflammation, necrosis and vasculitis predominantly affecting respiratory tract
Variable renal involvement
Predominantly C-ANCA positive (75%)
42
Churg-Strauss syndrome
```
Granulomatous inflammation
Peripheral blood eosinophilia
Can have asthmatic symptoms
60% P-ANCA, 10% C-ANCA, 30% negative
Systemic disease - may get kidney damage, may diagnose from biopsy
```
43
List 7 small vessel vasculitides
Microscopic polyangiitis
Wegener's granulomatosis
Churg-Strauss syndrome
Henoch-Schonlein purpura (IgA deposition)
Cryoglobulinaemic vasculitis (test for cryoglobulins)
Serum sickness
Rheumatoid and lupus vasculitis (Rh factor, anti-dsDNA)
44
List 3 manifestations of small vessel vasculitis
Nephritis: usually a necrotising and crescentic GN
Pulmonary haemorrhage: haemorrhagic alveolar capillaritis, heals as interstitial fibrosis
Pulmonary-renal syndrome: in microscopic polyangiitis and Goodpastures' syndrome
Skin: purpura (non-blanching, more common in lower limbs)
Abdominal pain: bowel necrosis, ulceration and haemorrhage, ??
Peripheral neuropathy, myalgia, arthralgia: involvement of small arteries and arterioles, may have elevated muscle enzymes
45
Microscopic polyangiitis
Necrotising systemic vasculitis affecting arterioles, capillaries and venules (and sometimes small and medium sized arteries)
Characterised by ??
Segmental vascular necrosis
Infiltrate of neutrophils and monocytes
May have leukocytoclasis - split neutrophils?, fibrin and haemorrhage
Thrombosis in small arteries causing infarction
Healing with fibrosis
Pseudoaneurysms may occur in medium sized arties)
46
Churg-Strauss syndrome
```
Granulomatous inflammation
Peripheral blood eosinophilia
Can have asthmatic symptoms
60% P-ANCA, 10% C-ANCA, 30% negative
Systemic disease - may get kidney damage, may diagnose from biopsy
```
47
Henoch-Schonlein purpura
??
48
Medium-sized vasculitides
Polyarteritis nodosa
49
Polyarteritis nodosa
Ag often unknown (sometimes Hep B/C)
| ??
50
Microscopic polyangiitis vs PAN
??
51
Temporal artery - external carotid system - why blindness?
Potentially internal carotid system leading to retinal artery could also be affected
52
Temporal artery - external carotid system - why blindness?
Indicates that potentially internal carotid system leading to retinal artery could also be affected
53
Temporal artery - external carotid system - why blindness?
Indicates that potentially internal carotid system leading to retinal artery could also be affected
54
Temporal arteritis
Medical emergency - steroids?
55
Temporal arteritis
Medical emergency - steroids?
56
List ? causes of haemopericardium
?
57
List ? causes of haemopericardium
?
58
How does haemopericardium lead to death of a patient?
When the pericardial pressure exceeds the intra-chamber pressures, the chamber will collapse
CO and systemic BP drops
59
What are the potential complications of aortic dissection?
```
Cardiac tamponade
Aortic incompetence
MI
Aneurysm rupture
Regional ischaemia
Left arm ischaemia/vertebral steal
```
60
What abnormalities of the vessel wall are seen in Marfan's syndrome?
?
61
List 5 causes of mitral valve incompetence
Infective endocarditis
Acute rheumatic fever
Ischaemic papillary muscle dysfunction or rupture
LV dilation
Myxomatous degeneration of leaflets or chordae
62
How is Marfan's syndrome inherited? What % of cases are sporadic?
AD
| Up to 30%
63
What is the abnormality in Marfan's syndrome?
Defect in fibrillin-1 gene, a major building block of microfibrils and important in formation and maintenance of elastin in the ECM
Microfibrils also help regulated TGF-B availability, leading to aberrant activation in Marfan's which is believed to have effects on the integrity of the ECM
64
List ? manifestations of Marfan's syndrome
Mitral valve prolapse/myxomatous degeneration
Cystic medionecrosis (cystic medial degeneration) of aorta
Spontaneous pneumothorax
Lens dislocation (ectopia lentis)
65
List ? manifestations of Marfan's syndrome
Mitral valve prolapse/myxomatous degeneration
Cystic medionecrosis (cystic medial degeneration) of aorta
OTHER?
66
What are the main risk factors for aortic dissection?
```
Marfan's syndrome
Ehlers-Danlos syndrome
Biscuspid aortic valve
Aortic atherosclerosis
Aortic dilation
Cardiac risk factors (HTN, dyslipidaemia, DM, smoking)
```
67
List ? clinical manifestations of Marfan's syndrome
Mitral valve prolapse/myxomatous degeneration
Cystic medionecrosis (cystic medial degeneration) of aorta
OTHER?
68
How might mitral valve prolapse present?
Asymptomatic
Mitral incompetence +/- ruptured chordae
Arrhythmias and sudden death
Predisposition to infective endocarditis
69
Diagnosis of vasculitis
Clinical features
Serology: ANCA, cryoglobulins, Hep B and C, complement, ANAs and rheumatoid factor
Pathology: biopsy if possible
70
List some potential causes of sudden collapse and death in a young adult
?
71
List ? causes of mitral (aortic??) valve incompetence
Marfan's syndrome? dissecting aortic aneurysm?
72
List ? causes of haemopericardium
?
73
How does haemopericardium lead to death of a patient?
?
74
What are the main risk factors for aortic dissection?
?
75
What are the potential complications of aortic dissection?
?
76
What abnormalities of the vessel wall are seen in Marfan syndrome?
?
77
List ? causes of mitral valve incompetence
Marfan syndrome? dissecting aortic aneurysm?
78
How is Marfan syndrome inherited? What % of cases are sporadic?
AD
| Up to 30%
79
What is the abnormality in Marfan syndrome?
Defect in fibrillin-1 gene, a major building block of microfibrils and important in formation and maintenance of elastin in the ECM
Microfibrils also help regulated TGF-B availability, leading to aberrant activation in Marfan's which is believed to have effects on the integrity of the ECM
80
What is the abnormality in Marfan syndrome?
Defect in fibrillin-1 gene, a major building block of microfibrils and important in formation and maintenance of elastin in the ECM
Microfibrils also help regulated TGF-B availability, leading to aberrant activation in Marfan's which is believed to have effects on the integrity of the ECM
81
List ? manifestations of Marfan syndrome
Mitral valve prolapse/myxomatous degeneration
Cystic medionecrosis (cystic medial degeneration) of aorta
OTHER?
82
How might mitral valve prolapse present?
Asymptomatic
Mitral incompetence +/- ruptured chordae
Arrhythmias and sudden death
Predisposition to infective endocarditis
83
List ? clinical manifestations of Marfan syndrome
Mitral valve prolapse/myxomatous degeneration
Cystic medionecrosis (cystic medial degeneration) of aorta
OTHER?
84
How might mitral valve prolapse present?
Asymptomatic
Mitral incompetence +/- ruptured chordae
Arrhythmias and sudden death
Predisposition to infective endocarditis
85
What are some of the possible sequelae of cystic medionecrosis?
Aortic dissection
| Annuloaortic ectasia and aortic regurgitation
86
What are the main risk factors for aortic dissection?
?
87
List ? causes of mitral (aortic??) valve incompetence
Marfan syndrome? dissecting aortic aneurysm?
88
Describe the likely pathogenesis of retinal flame haemorrhages
?
89
Describe the likely pathogenesis of retinal flame haemorrhages
?
90
What are the potential causes of systemic HTN in a 32 year old?
?
91
What are the potential causes of systemic HTN in a 32 year old?
?
92
What are the potential causes of a spontaneous SAH?
?
93
What are the potential causes of a spontaneous SAH?
?
94
How should a SAH be managed?
BP control
Analgesia
Find the cause, intervene neurosurgically
95
What is the pathogenesis of vasospasm post-SAH? What can it cause?
?
96
What are the 3 elements of the Cushing reflex?
HTN
Bradycardia
Irregular breathing
97
What are the 3 elements of the Cushing reflex?
HTN
Bradycardia
Irregular breathing
98
List ? causes of SAH
?
99
What are the risk factors for formation of berry aneurysms?
?
100
Where are the most common sites for berry aneurysms?
?
101
What are the presenting clinical features of SAH?
?
102
What are the complications of SAH?
?
103
What proportion of patients with SAH die?
?
104
Name 3 other types of aneurysms that may occur in vessels supplying the CNS
?
105
List ? potential causes of renal and splenic infarction
?
106
List 4 histological findings associated with vasculitis
Fibrinoid necrosis
Thrombosis
Perivascular haemorrhage
Leukocytoclasis
107
What symptoms are consistent with vasculitis?
Range of clinical manifestations depending on affected organs
Constitutional symptoms of fever, LOW, malaise, arthralgias and myalgias
108
List the 4 causes of vasculitis
Immune mechanisms (deposition of circulating immune complexes or in situ formation of immune complexes following previous seeding of Ag in the vascular bed)
Infection (direct invasion and immune-mediated)
Radiation
Toxins, drugs
109
List the 4 causes of vasculitis
Immune mechanisms (deposition of circulating immune complexes or in situ formation of immune complexes following previous seeding of Ag in the vascular bed)
Infection (direct invasion and immune-mediated)
Radiation
Toxins, drugs
110
List 2 vasculitides predominantly affecting large vessels
Giant cell arteritis
| Takayasu arteritis
111
List 2 vasculitides predominantly affecting medium vessels
Polyarteritis nodosa
| Kawasaki disease
112
List 3 ANCA-associated vasculitides predominantly affecting small vessels
Microscopic polyangiitis
Wegener's granulomatosis
Churg-Strauss granulomatosis
113
List 3 ANCA-associated vasculitides predominantly affecting small vessels
Microscopic polyangiitis
Wegener's granulomatosis
Churg-Strauss granulomatosis
114
List 4 immune complex vasculitides predominantly affecting small vessels
Anti-GBM disease (Goodpasture's)
Cryoglobulinaemic vasculitis
IgA vasculitis (Henoch-Schonlein)
Hypocomplementemic urticarial vasculitis
115
List 4 immune complex vasculitides predominantly affecting small vessels
Anti-GBM disease (Goodpasture's)
Cryoglobulinaemic vasculitis
IgA vasculitis (Henoch-Schonlein)
Hypocomplementaemic urticarial vasculitis
116
Distinguish between ANCA-associated and immune complex mediated vasculitides
ANCA-associated: few or no immune deposits, associated with ANCA specific for myeloperoxidase (APO-ANCA) or proteinase 3 (PR3-ANCA)
Immune complex mediated: moderate to marked deposits of Ig and/or complement in vessel walls
117
Distinguish between ANCA-associated and immune complex mediated vasculitides
ANCA-associated: few or no immune deposits, associated with ANCA specific for myeloperoxidase (APO-ANCA) or proteinase 3 (PR3-ANCA)
Immune complex mediated: moderate to marked deposits of Ig and/or complement in vessel walls
118
List 3 vasculitides associated with systemic disease
Lupus
Rheumatoid
Sarcoid
119
List 3 vasculitides associated with systemic disease
Lupus
Rheumatoid
Sarcoid
120
List 6 causes/associations of small vessel vaculitides
```
AI
Associated with other systemic disease
Infections (e.g. hep B, hep C)
Malignancy
Drugs
Idiopathic/unknown
```
121
List 6 causes/associations of small vessel vaculitides
```
AI
Associated with other systemic disease
Infections (e.g. hep B, hep C)
Malignancy
Drugs
Idiopathic/unknown
```
122
List 6 causes/associations of small vessel vaculitides
```
AI
Associated with other systemic disease
Infections (e.g. hep B, hep C)
Malignancy
Drugs
Idiopathic/unknown
```
123
Describe the likely pathogenesis of retinal flame haemorrhages
?
124
List 4 potential causes of a spontaneous SAH
Saccular aneurysm rupture
AV malformations
Arterial dissections
Use of anticoagulants
125
What is the pathogenesis of vasospasm post-SAH? What can it cause?
Believed to result from a delayed and reversible vasculopathy, impaired autoregulatory function and hypovolaemia
Can progress to cerebral infarction
126
What are the presenting clinical features of SAH?
```
Headache
Photophobia
Nausea/vomiting
Altered mental state
LOC
```
127
What are the complications of SAH?
```
Rebleeding
Acute hydrocephalus
Vasospasm
Pulmonary oedema
Hyperglycaemia
Seizure
Death
```
128
What proportion of patients with SAH die?
Up to 50%
129
Name 3 other types of aneurysms that may occur in vessels supplying the CNS
Fusiform
Dissecting/pseudo-aneurysm
Charcot-Bouchard (micro-) aneurysms
