Large Vessel Vasculitis Flashcards by Caitlin McMullen

What is the pathophysiology of vasculitis?

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

Often autoimmune

How well did you know this?

Not at all

Perfectly

What is a key caution regarding ANCA testing in vasculitis?

ANCA is not a diagnostic test for all types of vasculitis

How well did you know this?

Not at all

Perfectly

What are the potential consequences of poor management of vasculitis?

Permanent organ damage

How well did you know this?

Not at all

Perfectly

What is the primary feature of large vessel vasculitis?

Chronic granulomatous inflammation predominantly affects the
1. aorta
2. its major branches

How well did you know this?

Not at all

Perfectly

What arteries are affected in Takayasu arteritis?

Arteries branching from the aortic arch

How well did you know this?

Not at all

Perfectly

What is the typical demographic for Takayasu arteritis?

(1) It occurs in individuals <40 years old.

(2) It is more common in females and Asian populations.

How well did you know this?

Not at all

Perfectly

What are the late clinical features of Takayasu arteritis?

(1) Claudication in arms
(2) vascular stenosis
(3) aneurysms, bruit
(commonly carotid)

(4) reduced pulses
(5) blood pressure differences between extremities

How well did you know this?

Not at all

Perfectly

What investigations are used for Takayasu arteritis?

First line - CT scan/ MRI angiography
ESR/CRP would be raised

Confirm - confirmed by vascular imaging, not biopsy

How well did you know this?

Not at all

Perfectly

What arteries are affected in Giant Cell Arteritis?

The carotid artery and its branches

How well did you know this?

Not at all

Perfectly

What is the typical demographic for Giant Cell Arteritis?

It occurs in individuals >50 years old (most commonly late 60s).
It is more common in females.
Strong association with polymyalgia rheumatica

How well did you know this?

Not at all

Perfectly

What are the symptoms of Giant Cell Arteritis?

(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

How well did you know this?

Not at all

Perfectly

What are the signs of giant cell arteritis?

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

(2) Carotid bruit

How well did you know this?

Not at all

Perfectly

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

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

How well did you know this?

Not at all

Perfectly

What is the gold standard for investigating giant cell arteries?

Temporal artery biopsy

How well did you know this?

Not at all

Perfectly

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

Prednisolone 40–60 mg daily

How well did you know this?

Not at all

Perfectly

What additional medications may be prescribed alongside steroids?

(1) Bisphosphonates (eg, Alendronic acid) to prevent bone loss.

(2) PPIs to prevent GI side effects.

(3) Low-dose aspirin to reduce stroke or blindness risk.

How well did you know this?

Not at all

Perfectly

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

methotrexate or leflunomide

How well did you know this?

Not at all

Perfectly

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

High-dose IV methylprednisolone

How well did you know this?

Not at all

Perfectly

Giant Cell Arteritis (GCA) can lead to what?

Ischaemic stroke

How well did you know this?

Not at all

Perfectly

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?

Takayasu’s arteritis

How well did you know this?

Not at all

Perfectly

Confirmation of the diagnosis of giant cell arteries is what?

vascular ultrasound and temporal artery biopsy

How well did you know this?

Not at all

Perfectly

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

12-18 months later

How well did you know this?

Not at all

Perfectly

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?

Erythrocyte sedimentation rate (ESR)

= most sensitive test to diagnose this condition

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

Halo sign

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

corticosteroid use - shingles

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 (1) ESR 76 mm/hr (<20 mm/hr) (2) 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?

Temporal arteritis

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?

Temporal artery biopsy

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?

steroids

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) ?

as there is pain and stiffness in both shoulders and hips

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?

ESR

When is Temporal artery biopsy used?

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

When is a Temporal artery ultrasound used?

used in the evaluation of patients with suspected Giant cell arteritis = does not confirm and is not first-line

Patient has visual loss, what drug would you commence

A 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?

Prednisolone

What condition is associated with Giant Cell Arteritis?

Polymyalgia Rheumatica

How does giant cell arteritis present?

1. Jaw claudication 2. Headache 3. Scalp tenderness 4. Visual loss

What is the affected artery in Giant Cell Arteritis?

Temporal artery

What is the treatment of giant cell arteritis with visual symptoms?

IV methylprednisolone

What is the immediate management for suspected giant cell arteritis?

High dose prednisolone (60mg OD)

If giant cell arteritis (GCA) is suspected, why should it be treated as such even if a temporal artery biopsy is negative?

Temporal artery biopsy is the gold standard investigation to diagnose GCA, but this may be normal if a "skip lesion" is biopsied and so cannot be relied upon

Which blood test is used to screen for Temporal arteritis?

Raised ESR

Why does a normal biopsy of the temporal artery not exclude Giant Cell Arteritis?

The vasculitis can affect the temporal artery in ‘skip lesions’

Why do patients need to receive high-dose corticosteroids in Giant Cell Arteritis?

To prevent irreversible sight loss

A 64-year-old woman presents to A&E with a severe temporal headache. Over the last 48 hours, she has been noticing pain in her jaw as she chews. Her past medical history includes hypertension, hypercholesterolaemia and type 2 diabetes. She denies visual symptoms. What is the most appropriate first-line treatment?

High-dose oral Prednisolone, once daily

An 80-year-old woman presents to her GP with a one-day history of a constant severe left-sided headache and pain in her jaw. She also has new blurred vision. The GP strongly suspects temporal arteritis and refers her to the hospital where is given a course of IV methylprednisolone and is subsequently maintained on high-dose oral prednisolone. Which other medication should also be prescribed?

Alendronic acid = A bisphosphonate and a proton pump inhibitor

When should oral vs. IV steroids be used in GCA?

visual disturbances = IV presence of a raised ESR (or CRP) and no visual disturbances = oral

A 68-year-old man presents to his GP with a four-day history of a severe, right-sided headache and facial pain. He also reports recent pain in his jaw while eating. He denies any visual changes or nasal discharge. His medical history includes recurrent sinusitis and bilateral shoulder pain which is currently under investigation. On examination, his scalp is very tender on the right side. What is a complication of the most likely diagnosis?

Ischaemic stroke

Polymyalgia Rheumatica means what in questions

pain and stiffness present

A 68-year-old female patient attends A&E with a left-sided headache, jaw claudication, scalp tenderness and reduced visual acuity to counting fingers in the left eye. She has a markedly raised erythrocyte sedimentation rate (ESR) and CRP. On examination of the left fundus, you note a swollen, chalky white, optic disc. The right fundus is normal. What is the most likely cause of her visual symptoms?

Anterior ischemic optic neuropathy

When should ESR/CRP vs. temporal artery biopsy be used in suspected giant cell arteritis?

ESR/CRP first to assess inflammation Temporal biopsy is confirmatory but is done after starting steroids, as treatment shouldn’t be delayed.

What are the symptoms of Takayasu arteritis?

(1) absent upper extremity pulse (2) discrepancy in blood pressure between the arms (>10 mm Hg) (3) night sweats (4) arthritis (5) myalgia (6) skin nodules (7) visual defects (8) stroke

Which patient group is most commonly affected by Takayasu arteritis?

It is most common in Asian women between 10 and 40 years old

A 43-year-old woman visits the GP having felt unwell for two months. During this time she has lost 4kg and felt lethargic. She works as a shop assistant and gets cramping pain in her right arm when stacking shelves, this has been worsening. On examination, the right hand is cooler than the left, with an absent radial pulse. The right carotid pulse feels weak, and there is a bruit on auscultation. On examination of the heart, you hear an early diastolic murmur maximal at the right sternal border, 4th inter-costal space. What investigation is best to confirm the most likely diagnosis?

CT angiography = first choice diagnostic test for Takayasu’s

"pulseless disease" What disease is known as this?

Takayasu arteritis

A 62-year-old man presents to the Emergency Department with a sudden onset headache and pain in his jaw whilst he was chewing breakfast in the morning. High-dose prednisolone is commenced and an appropriate biopsy is performed which later comes back to normal. What has happened in this case?

Skip lesions occur in giant cell arteritis and may show a normal biopsy therefore continue prednisolone

In a fundoscopy what would you see for anterior ischemic optic neuropathy?

swollen pale disc and blurred margins

A 67-year-old man presents to the general practitioner with a 2-day history of visual loss in his right eye. There is no past medical history of note, although he reports taking regular co-codamol for intermittent headaches. On examination, his observations are within normal limits. Visual acuity in the right eye is 6/30 in the right eye and 6/6 in the left eye. A fundoscopic examination of the right eye shows a swollen and pale optic disc with blurred margins. What is the most likely diagnosis?

Anterior ischaemic optic neuropathy anterior

Large Vessel Vasculitis Flashcards

Giant cell Vasculitis + Takayasu arteritis