Large-vessel vasculitis: Takayasu arteritis, Giant Cell Arteritis

Question 1

Define vasculitis?

Answer 1

Heterogeneous group of conditions characterised by blood vessel wall inflammation

Question 2

What are the 2 most common pathophysiologies of vasculitis?

Answer 2

Immune-mediated inflammation

Direct vascular pathogenic invasion

Question 3

Apart from immune inflammation and pathogenic invasion, how can physical and chemical injuries to vessel walls cause vasculitis?

Answer 3

Factors such as mechanical trauma, toxins, radiation

Question 4

Give the 3 primary ways of classifying vasculitides?

Answer 4

Vessel diameter

Involvement of immune complexes

ANCA-associated or not

Question 5

Define granulomatous vasculitis?

Answer 5

Pathological process due to the destruction of vessels by a granulomatous infiltrate which contains different immune cell lines

Question 6

Is Takayasu arteritis a type of granulomatous or non-granulomatous vasculitis?

Answer 6

Granulomatous

Question 7

Which 3 blood vessels are characteristically inflamed in Takayasu arteritis?

Answer 7

Aorta

Major aortic branches

Medium-sized pulmonary arteries

Question 8

How does Takayasu arteritis characteristically affect the upper extremities?

Answer 8

Causes weak pulse and bp

Question 9

Which facial feature does Takayasu arteritis characteristically affect?

Answer 9

Eyes

Causes ocular disturbances

Question 10

What is the ratio of females to males who develop Takayasu arteritis?

Answer 10

8:1

Much more prevalent in females

Question 11

Which continent has the highest prevalence and incidence of Takayasu arteritis?

Answer 11

Asia

Question 12

What is the typical onset age range for Takayasu arteritis?

Answer 12

25-40

Question 13

In Takayasu arteritis, what is the first stage of progression, and which vessel does it occur in?

Answer 13

Scarring and thickening of aorta, arch branches due to granulomatous inflammation causes significant lumen narrowing

Question 14

In the pre-pulseless/early symptoms of Takayasu arteritis, are symptoms specific or systemic?

Answer 14

Systemic symptoms

eg. weight loss, arthragia, fever, fatigue, claudication (muscle pain due to lack of oxygen that’s triggered by activity and relieved by rest)

Question 15

Define claudication, and in which phase of Takayasu arteritis does it occur?

Answer 15

Muscle pain due to lack of oxygen that’s triggered by activity and relieved by rest

Early symptom/pre-pulseless phase

Question 16

In which phase of Takayasu arteritis do ocular disturbances occur, and give 3 common examples?

Answer 16

Late symptom/pulseless phase

eg. visual field defects, retinal defects, blindness

Question 17

In which phase of Takayasu arteritis does weak pulse and bp in upper extremities occur?

Answer 17

Late symptoms/pulseless phase

Question 18

Give 2 common symptoms of pulmonary artery involvement in Takayasu arteritis, and which phase does this occur in?

Answer 18

Pulmonary hypertension

Bruits/vascular murmurs (sound caused by turbulent blood flow of blood in artery, possibly due to partial obstruction)

Occur in late symptoms/pulseless phase

Question 19

What type of x-ray is done to investigate Takayasu arteritis?

Answer 19

Angiography: X-ray of blood vessels

Question 20

What is meant by occlusion?

Answer 20

complete/partial blockage of blood vessel

Question 21

Define aneurysmal dilation, and what is the other name for this?

Answer 21

Aortic root aneurysm

enlargement/bulging near aortic valve due to weakened vessel walls

Question 22

Define coarctation?

Answer 22

Narrowing in aortic arch

Question 23

What are the 3 characteristic radiological findings on an angiogram, in Takayasu arteritis?

Answer 23

Coarctation (narrowing in aortic arch)

Aortic root aneurysm (Bulging near aortic valve due to weakened walls)

Occlusion (complete/partial blockage of blood vessel)

Question 24

What lab test is done to diagnose Takayasu arteritis, and what is the characteristic finding?

Answer 24

FBC

Normocytic normochromic anaemia: Low number of normal-sized (normocytic) and normal red-coloured (normochromic) RBCs

Question 25

Define normocytic normochromic anaemia?

Answer 25

Low number of normal-sized (normocytic) and normal red-coloured (normochromic) RBCs

Question 26

How is Takayasu arteritis generally managed?

Answer 26

High-dose glucocorticoids and immunosuppressants

Question 27

Do people with Takayasu arteritis have a high or low 5-year survival rate?

Answer 27

High (83%)

Question 28

Are people with Takayasu arteritis likely to make a full recovery in the long-term?

Answer 28

No, they are more likely to have visual, neurological deficits

Question 29

What are the other 2 names for Giant Cell Arteritis (GCA)?

Answer 29

Temporal arteritis Cranial arteritis

Question 30

Is GCA a type of granulomatous or non-granulomatous vasculitis?

Answer 30

Granulomatous

Question 31

Which arteries are characteristically inflamed in GCA?

Answer 31

Arteries in head, mostly temporal arteries (around temples) and occipital arteries (posterior portion of neck and back of head)

Question 32

Which other rheumatological condition is GCA commonly associated with?

Answer 32

Polymyalgia rheumatica

Question 33

Give 2 of the largest risk factors for developing GCA?

Answer 33

Old age North-European descent

Question 34

How does age affect risk of developing GCA?

Answer 34

Risk increases with age

Question 35

How does ethnic descent affect risk of developing GCA?

Answer 35

North-European descent is at higher risk

Question 36

What is the typical onset age for GCA?

Answer 36

Over 50 yrs old

Question 37

In GCA presentation, describe the person's energy level?

Answer 37

Low energy, feel fatigued

Question 38

In GCA presentation, where does facial pain tend to occur and which 2 movements exacerbate it?

Answer 38

Jaw claudication Worsened by chewing and talking

Question 39

In GCA presentation, where are headaches most intense and tender upon palpation and why?

Answer 39

Around temples Because temporal arteries are there

Question 40

How does GCA commonly affect the scalp in 3 ways?

Answer 40

Tender scalp Hair loss Red, shiny ulcers

Question 41

How does GCA commonly cause temporal and occipital arteries to look and feel?

Answer 41

Thickened and bulging from skin Tender, low pulsation

Question 42

Is GCA due to immune-mediated inflammation or vascular pathogenic invasion?

Answer 42

T-cell mediated immune response to antigen found in vessel wall leads to granulomatous inflammation and high interleukin levels

Question 43

Which artery is affected in GCA, to cause ocular problems?

Answer 43

Ophthalmic artery

Question 44

Is vision loss or vision disturbance more common in GCA?

Answer 44

Vision disturbance in 25-50% cases Vision loss in 6-10% cases

Question 45

Give 4 examples of ocular problems that occur due to ophthalmic artery involvement in GCA?

Answer 45

Diplopia (double vision) Visual hallucinations Temporary monocular blindness Complete blindness

Question 46

How does GCA affect the risk of aneurysms, and where are they most likely to occur?

Answer 46

Increases risk of aneurysms Most likely to be aortic aneurysms

Question 47

Give 2 ways in which GCA can cause serious heart complications?

Answer 47

Aortic aneurysm Myocardial infarction

Question 48

How does GCA affects the risk of strokes?

Answer 48

Increases risk of cerebral vascular accidents (strokes)

Question 49

What used to be the first-line investigation for diagnosing GCA, and why is it not first-line anymore?

Answer 49

Temporal artery biopsy Negative biopsy result doesn’t rule out GCA as vascular inflammation is patchy and the non-inflamed tissue area could have been obtained for the sample instead

Question 50

Why does a single negative temporal artery biopsy not rule out GCA, and how can you be more sure to rule out GCA using this investigation?

Answer 50

Negative biopsy result doesn’t rule out GCA as vascular inflammation is patchy and the non-inflamed tissue area could have been obtained for the sample instead Should obtain multiple samples to biopsy

Question 51

What is the current gold-standard for investigating GCA, and what is the characteristic sign?

Answer 51

Ultrasound imaging shows 'halo' sign ‘halo’ sign: dark thick border (edema/swelling in thickened vessel wall) surrounding narrowed arterial lumen

Question 52

When would you use PET scan to investigate GCA, and what is the main disadvantage?

Answer 52

When GCA affects deep vessels Uses large amount of radiation

Question 53

Describe the CRP and ESR levels, in GCA?

Answer 53

Elevated

Question 54

Describe the 2 main findings of a FBC, in GCA?

Answer 54

Elevated platelet count Normochromic normocytic anaemia

Question 55

Why is GCA a medical and ophthalmic emergency?

Answer 55

Causes complete blindness (as ophthalmic artery vasculitis can lead to sudden and permanent blindness) Other life-threatening complications due to occlusion/rupture of arteries

Question 56

If someone has GCA and suspected blindness, what medication should you prescribe to have an effect within 48-72 hrs, and what is the starting dose?

Answer 56

Prednisolone 60-80 mg (weight-dependent)

Question 57

In GCA treatment, what should the dose of prednisolone ideally be after 8 weeks of administration?

Answer 57

Should be tapered down from 60-80mg to 10-15mg, after 8 weeks

Question 58

After the prednisolone dose has been reduced to 10-15 mg, how should it be further tapered and adjusted if there is a flare up, in GCA?

Answer 58

Now rate of reduction should decrease by 1 mg per month, if flare occurs then increase dosage again and try tapering in few weeks time

Question 59

To treat resistant cases of GCA, what medication is now used and for how long?

Answer 59

Tocilizumab can be used for resistant cases for 12 months

Question 60

When can bisphosphonates be taken to help treat GCA?

Answer 60

After assessing risk of osteoporosis and vitamin D sufficiency