Aneurysms & Vasculitides Flashcards

1
Q

What are aneurysms?

A

Abnormally dilated part of a vessel or the heart.

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2
Q

What causes aneurysms?

A

Congenital or acquired weakness in tunica media of the vessel (or the myocardium)

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3
Q

Where do aneurysms typically occur?

A

Usually in arteries or left ventricle

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4
Q

What are true and false aneurysms?

A

True aneurysm is an aneurysm in which the entire tissue is enlarged outward.

False anerysm is an aneurysm in which there is a hole in the vascular wall and leakage of blood to the outside

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5
Q

How are aneurysms calssified?

A

Cause (congenital, mycotic, inflammatory, traumatic arterial injury)

Morphology (Sacular, bubble-like outpouching or fusiform)

Anatomical site (Aortic, Iliac, Left ventricular, Popliteal, etc)

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6
Q

What type of aneurysms are typically seen in the abdominal aorta?

A

Fusiform

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7
Q

Where are berry aneurysms typically seen?

A

Circle of willis

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8
Q

What are the complications associated with aneurysms?

A

Rupture can lead to rapid loss of blood or sub-arachnoid haemorrhage

Occlusion can disturb the bloodflow in the vessel and can lead to thrombus formation which can occlude the involved vessel or one of its branches.

Mass effect which is the compression of surrounding structures like a tumour

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9
Q

What are cerebral saccular aneurysms?

A

Commonly called berry aneurysms. They are located at branch points of the large intracranial arteries in the circle of willis

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10
Q

Why do berry aneurysms form?

A

Weakened areas where they occur are caused by congenital defects in the smooth muscle layer of the artery (tunica media)

THE ANEURYSM ITSELF IS NOT CONGENITAL

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11
Q

What conditions cause increase risk in aneurysm formation?

A

Polycystic kidney disease and Ehlers-Danlos syndrome (conditions of the connective tissue)

Haemodynamic stress conditions such as hypertension and smoking cause aneurysm to expand over time.

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12
Q

Why is smoking a risk factor for aneurysms?

A

It damages arterial walls

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13
Q

What are complications of berry aneurysms?

A

Mass effects which cause compression of adjacent brain and cranial nerves which clinically leads to headaches, double vision, and loss of vision

Rupture can cause subarachnoid haemorrhage

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14
Q

What are the clinical features of berry aneurysms?

A

Before rupture:
Usually asymptomatic
Sentinel headache which can be severe
Vomiting, double vision, seizures (mass effect)

Rupture and subarachnoid haemorrhage:
Thunderclap headache
Vomiting, collapse, confusion, seizures
Approx 25% die within 24 hours
Further 25% die within 3 months
Of the survivors 50% have permanent neurological defects
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15
Q

How are berry aneurysms diagnosed?

A

MRI

CT scan

Angiogram

CSF analysis showing blood (=ggwpscrub)

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16
Q

When do aortic aneurysms occur?

A

Abnormality in tunica media -> destroys capacity of tissue to resist the haemodynamic forces of systole

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17
Q

What processes cause abnormalities in aortic tunica media?

A

Atherosclerosis

Non-inflammatory degeneration

Inflammation eg. syphilis, giant cell aortitis

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18
Q

Where on the aorta are aortic aneurysms most common?

A

Below the level of the renal arteries and above aortic bifurcation

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19
Q

Which people get AAA most often?

A

Caucasian, male, elderly

Risk factors include: Smokers, alcohol drinkers, hypertensive people and some genetic markers

Atherosclerosis causing medial atrophy

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20
Q

What are the possible complications of AAA?

A

Rupture causing large volume of blood under high pressure to escape into peritoneal cavity or the retroperitoneal tissues resulting in mass haemorrhage.

Peripheral thromboembolism causing occlusion of distal arteries

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21
Q

What are the clinical features of AAA?

A

Before rupture: Usually asymptomatic, can feel back pain, abdominal pain, and a throbbing sensation

Rupture: results in a triad of abdominal pain, hypovolaemic shock and a pulsatile abdominal mass.

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22
Q

How does aortic aneurysm rupture present itself?

A

Pain is sudden onset, excrutiating and is felt in the abdomen, flank or groin

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23
Q

How can AAA be diagnosed?

A

Clinical findings or imaging studies

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24
Q

What is the mortality rate of AAA?

A

90% mortality associated with AAA rupture

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25
Q

What are thoracic aneurysms associated with?

A

Hypertension, syphilis, heart failure secondary to aortic valve incompetence.

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26
Q

What are the clinical presentations of thoracic aneurysms?

A

Mass effect: airway obstruction, cough due to laryngeal nerve compression

Erosion of bone leading to pain

Aortic root issues (Aortic root dilation can result in aortic regurgitation) Narrowing of the coronary ostia.

May rupture leading to haemothorax and haemopericardium

May fistulate to lung or oesophagus.

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27
Q

What nerve causes excess coughing when compressed?

A

Laryngeal nerve

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28
Q

What does fistulate mean?

A

an abnormal or surgically made passage between a hollow or tubular organ and the body surface, or between two hollow or tubular organs.

29
Q

What are aortic dissections?

A

Blood enters the wall through a tear in the intima of the aorta. The blood then separates the media as it dissects a path along the length of a vessel.

30
Q

Are aortic dissections considered aneurysms?

A

No

31
Q

Where do thoracic dissections usually take place?

A

Within 2 cm of the aortic valve.

32
Q

What can happen to blood in thoracic dissections?

A

It can flow backwards towards the heart

It can flow back into aortic lumen

It can flow forwards forwards down the length of the aorta

33
Q

How do dissections happen in the aortic wall 2cm above the valve?

A

Pressure of blood forces a hole in the luminal side of the blood vessel resulting in a double lumen. Blood enters the false lumen and fills it up.

Dissection can result in tearing of outer wall and blood moving out into the pericardium causing cardiac arrest.

34
Q

Why do aortic dissections happen?

A

Weakening of tunica media of the aortic wall by either longstanding hypertension or by a congenital weakness such as found in marfan syndrome.

Chest trauma can cause aortic dissection

Surgery can cause aortic dissection

More common in men (50 - 70 years of age) than women

35
Q

What is cystic medial necrosis?

A

In cases of longstanding hypertension or marfan syndrome there is a change in the aortic wall known as “cystic medial necrosis”.: There are fewer elastic fibers in the tunica media as well as excess mucopolysaccharides, this is why aortas are less elastic in people with chronic HT.

36
Q

What are possible complications of aortic dissection?

A

Rupture: Blood can track into pericardium (cardiac tamponade), pleura, or peritoneum with massive and often fatal haemorrhage

Extension to branches of the aorta/occlusion: To coronary arteries would result in myocardial infarction.

To carotid arteries would cause stroke.

To aortic valve ring, causing marked aortic insufficiency

37
Q

What are the clinical features of an aortic dissection?

A

Sudden onset of excrutiating chest pain, or pain in the back between shoulder blades.

Tearing/stabbing type of pain

Collapse/shock

Risk of death is high (25% of people die within the first 24 hours and then 90% dead at one year if untreated)

38
Q

How is diagnosis of aortic dissection confirmed?

A

CXR - widened mediastinum

Transesophageal echo, CT scan

39
Q

What is marfan syndrome?

A

Autosomal dominant genetic disorder

Mutations of fibrillin gene

Abnormal elastic tissue

40
Q

What is vasculitis?

A

Inflammation of the blood vessels. (any blood vessels)

41
Q

How do blood vessels change in response to inflammation?

A

They either get thicker causing luminal narrowing / occlusion

Or they get thinner due to attenuation and form aneurysm.

42
Q

What are the types of primary vasculitides?

A

Giant cell arteritis

Takayasu’s arteritis

Kawasaki disease

43
Q

What are primary vasculitides?

A

Unique disease entities without a currently identified underlying cause where vasculites form pathological basis of tissue injury

44
Q

What are secondary vasculitides?

A

Vasculitis occuring secondary to an underlying disease or exposure

45
Q

What are some causes for secondary vasculitides?

A

Medications/drugs

Infection

Malignancy

Transplant (part of transplant process)

Cryoglobulinemia

Connective tissue disease

46
Q

What causes vasculitides?

A

Immune complex mechanisms activating the complement cascade

Direct attack on vessel wall by circulating antibodies

Cell mediated immune response is also possible

47
Q

How are vasculitides classified?

A

Aetiology (syphilitic aortitis is caused by infection)

Size of affected vessel

Location of the vessels affected

48
Q

What are some vasculitis diseases that affect the aorta?

A

Giant cell vasculitis

Takayasu’s arteritis

49
Q

What are some conditions that affect medium sized arteries?

A

Kawasaki’s disease (coronary artery aneurysm)

50
Q

What blood vessels does good pasture syndrome affect?

A

Capillaries

51
Q

How are vasculitides classified with size?

A

Large arteries (Takayasu, giant cell)

Medium sized arteries (Polyarteritis nodosa, kawasaki’s disease)

Small vessels (Wegener’s granulomatosis, Churg Strauss, Leukocytoclastic vasculitis, Henoch Shonlein purpura)

52
Q

What is temporal arteritis?

A

Inflammation of large and medium sized arteries.

Usually involves branches of the carotid and temporal artery

53
Q

What is diagnostic about giant cell vasculitis?

A

The cells are giant… duh

54
Q

What is the gold standard for giant cell arteririts diagnosis?

A

Biopsy of temporal artery

55
Q

What is polyarteritis nodosa?

A

A multisystem necrotising vasculitis involving medium sized arteries. It classically involves the renal arteries and the mesenteric arteries.

Can also involve the heart liver and skin.

Does NOT involve pulmonary arteries.

Focal and segmental artery involvement

Neutrophils lymphocytes and plasma cells enter arterial wall

Fibrinoid necrosis of the arterial wall

Narrowing of the lumen, thrombosis, and ischaemia of the tissues supplied by the artery.

56
Q

What is granulomatosis and polyangiitis?

A

Small vessel vasculitis

Affects upper and lower respiratory tract

57
Q

What is fibrinoid necrosis?

A

Death of cells in tissue with fibrin scarring.

58
Q

What is granulomatosis with polyangiitis?

A

Small vessel vaculitis

59
Q

Where is granulomatosis with polyangiitis typically found?

A

In the upper and lower respiratory tract and the kidney.

60
Q

What is the type of vasculitis in Wegner’s granulomatosis?

A

Necrotising and granulomatous

61
Q

What is another name for Wegener’s granulomatosis?

A

Granulomatosis with polyangiitis

62
Q

When does wegner’s granulomatosis typically happen?

A

Any age in anyone

63
Q

What is Wegner’s granulomatosis associated with?

A

ANCA, mostly c-ANCA

64
Q

What are granulomas?

A

Inflammatory foci with lots of granulomatic cells specifically macrophages. Necrotising granulomas can be seen.

65
Q

What condition is Wegener’s granulomatosis similar to?

A

Similar to TB but TB has more necrotising granulomas. There is hardly any necrosis in Wegener’s granulomatosis. The granulomas are not necrotic themselves but they cause necrosis.

In the lung an abscess like structure can form causing necrosis.

66
Q

What are possible consequences of Wegener’s granulomatosis?

A

Proteinuria

Haematuria

Renal failure

67
Q

What is another name for Wegener’s granulomatosis?

A

Granulomatosis with polyangiitis

68
Q

Why is the tunica media typically broken in aneurysms?

A

Because it is the most important layer of tissue that contains the elastin tissue. Chronic hypertension leads to injury in this layer which is most important.

69
Q

Are aortic dissections the same as false aneurysms?

A

No, they are not aneurysms at all.