Large Vessel Vasculitis Flashcards

1
Q

What is vasculitis?

A

Inflammation of blood vessels, including arteries, arterioles, veins, venules, and capillaries

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2
Q

What is the aetiology of primary vasculitis?

A

Inflammation of vessel walls with no known cause

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3
Q

What is the aetiology of secondary vasculitis?

A

Triggered by infections, drugs, toxins, other inflammatory disorders, or cancer

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4
Q

What is the pathophysiology of vasculitis?

A

Inflammation of blood vessels can lead to ischaemia, tissue necrosis, and organ damage.

Often autoimmune

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5
Q

What systemic symptoms are common to all types of vasculitis?

A

Fever, malaise, weight loss, and fatigue

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6
Q

What factors determine the clinical presentation of vasculitis?

A

The size and location of the affected vessels.

The degree of organ dysfunction and inflammation

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7
Q

What is a key caution regarding ANCA testing in vasculitis?

A

ANCA is not a diagnostic test for all types of vasculitis

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8
Q

What are the potential consequences of poor management of vasculitis?

A

Permanent organ damage

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9
Q

Is vasculitis a single disease?

A

nope

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10
Q

What is the primary feature of large vessel vasculitis?

A

Chronic granulomatous inflammation predominantly affecting the aorta and its major branches

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11
Q

What arteries are affected in Takayasu arteritis?

A

Arteries branching from the aortic arch

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12
Q

What is the typical demographic for Takayasu arteritis?

A

It occurs in individuals <40 years old.

It is more common in females and Asian populations.

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13
Q

What are the early clinical features of Takayasu arteritis?

A

Non-specific symptoms like
(1) low-grade fever
(2) malaise
(3) night sweats
(4) weight loss
(5) arthralgia
(6) fatigue

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14
Q

What are the late clinical features of Takayasu arteritis?

A

(1) Claudication in arms
(2) vascular stenosis
(3) aneurysms, bruit (commonly carotid)
(4) reduced pulses
(5) blood pressure differences between extremities

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15
Q

What investigations are used for Takayasu arteritis?

A

Bloods: Raised inflammatory markers (ESR, CRP)

Imaging: MR/CT angiogram to detect thickened vessel walls and stenosis

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16
Q

What arteries are affected in Giant Cell Arteritis?

A

Carotid artery and its branches.

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17
Q

What is the typical demographic for Giant Cell Arteritis?

A

It occurs in individuals >50 years old (most commonly late 60s).

It is more common in females.

Strong association with polymyalgia rheumatica.

18
Q

What are the symptoms of Giant Cell Arteritis?

A

(1) Unilateral acute temporal headache with tenderness

(2) Jaw claudication (pain while chewing or speaking)

(3) Visual disturbances (blurring, anterior ischemic optic neuropathy, or blindness)

(4) Systemic symptoms: Fatigue, malaise, and fever

19
Q

What are the signs of giant cell arteritis?

A

(1) Tender, enlarged, non-pulsatile temporal arteries

(2) Carotid bruit

20
Q

What is a key diagnostic clue for GCA in older adults?

A

A new-onset headache in patients aged >50 with elevated ESR or CRP

21
Q

What is the gold standard for investigating giant cell arteries?

A

Temporal artery biopsy

22
Q

What is the first-line treatment for both Takayasu arteritis and Giant Cell Arteritis?

A

Prednisolone 40–60 mg daily

23
Q

What additional medications may be prescribed alongside steroids?

A

Bisphosphonates (e.g., Alendronic acid) to prevent bone loss.

PPIs to prevent GI side effects.

Low-dose aspirin to reduce stroke or blindness risk.

24
Q

What is the long-term management strategy for Takayasu arteritis?

A

methotrexate or leflunomide

25
Q

What is the emergency treatment for GCA with visual disturbances or complications?

A

High-dose intravenous steroids

26
Q

Giant Cell Arteritis (GCA) can lead to what?

A

Ischaemic stroke

27
Q

A 25-year-old female presents with new-onset headaches, vision changes, and arm claudication. On examination, there are weak radial pulses and a blood pressure difference of >10 mmHg between the arms.

What is the most likely diagnosis?

A

Takayasu’s arteritis

28
Q

Confirmation of the diagnosis of giant cell arteries is what?

A

vascular ultrasound and temporal artery biopsy

29
Q

When would low-dose Prednisolone, once daily be used instead of high dose?

A

12-18 months later

30
Q

A 79-year-old patient presents with a 6-day history of a left-sided headache and reduced visual acuity in her left eye. She also complains of pain when chewing.

She has a past medical history of polymyalgia rheumatica.

Which is a first-line investigation that will most likely reveal the possible underlying diagnosis? and why?

A

Erythrocyte sedimentation rate (ESR)

= most sensitive test to diagnose this condition

31
Q

What sign would most likely be present in a Doppler ultrasound test of the temporal arteries when investigating giant cell arteries?

A

Halo sign

32
Q

A painful rash in those with GCA is caused by what?

A

corticosteroid use - shingles

33
Q

A 68-year-old male complains of a unilateral headache and scalp tenderness. Additionally, he complains of stiffness in his shoulders, which is most noticeable in the morning. Blood tests reveal: ESR 76 mm/hr (<20 mm/hr), and creatine kinase 30 U/L (25 – 200 U/L). A temporal artery biopsy reveals no abnormality.

What is the most likely cause of this man’s headache?

A

Temporal arteritis

34
Q

A 65-year-old male presents to his GP with a 2-day history of a progressively worsening headache. He also reports pain whilst eating. On examination, his scalp is tender.

Given the most likely underlying diagnosis, what is the most appropriate investigation to confirm it?

A

Temporal artery biopsy

35
Q

A 79-year-old man presents to the GP with a 3-day history of epigastric pain that is accompanied by nausea and worse whilst eating. Urea breath test shows no detection of 13C carbon isotope.

His past medical history consists of hypertension, ischaemic heart disease and a recent discharge a week ago for giant cell arteritis. He has a 30-pack-year history but quit smoking five years ago and drinks 8 units of alcohol weekly.

What is the likely cause of his symptoms?

A

steroids

36
Q

A 62 year old woman presents with an acute onset severe headache which is on the right side and over the temporal region. She was unable to tolerate eating as she describes pain in her jaw when she eats. She is complaining of blurred vision. There is pain and stiffness in both her shoulders and hips. The temporal artery on the right side is thickened and tender to the touch. Bloods reveal a raised ESR and raised CRP. The admitting team organises an urgent biopsy of the temporal artery. The results come back as negative (normal findings).

Why Is the answer Giant Cell Arteritis (GCA) with Polymyalgia Rheumatica (PMR) and not just Giant Cell Arteritis (GCA) ?

A

as there is pain and stiffness in both shoulders and hips

37
Q

A 75-year-old woman on long-term steroids comes to A and E following a week of malaise. She thinks she has had a low-grade fever and has lost some weight recently. She complains of a right-sided headache and, on examination, has scalp tenderness on the right side.

What is the first line investigation you would order?

A

ESR

38
Q

When is Temporal artery biopsy used?

A

Temporal artery biopsy is the definitive test, that all patients with suspected giant cell arteritis should undergo. However, it is not ‘first line’ and treatment would not be delayed while awaiting biopsy in a patient with suspected giant cell arteritis

39
Q

When is a Temporal artery ultrasound used?

A

used in the evaluation of patients with suspected Giant cell arteritis

= does not confirm and is not first line

40
Q

Patient has visual loss, what drug would you commence

A

IV

41
Q

54 year 54-year-old female presents to her general practitioner with a 2 week history of left-sided headaches. It is worse when combing the left side of her head. Today, before her appointment, she described a transient visual defect in her left eye.

What should be the first course of action undertaken by the GP?

A

Prednisolone