Large Vessel Vasculitis Flashcards
Giant cell Vasculitis + Takayasu arteritis
What is vasculitis?
Inflammation of blood vessels, including arteries, arterioles, veins, venules, and capillaries
What is the aetiology of primary vasculitis?
Inflammation of vessel walls with no known cause
What is the aetiology of secondary vasculitis?
Triggered by infections, drugs, toxins, other inflammatory disorders, or cancer
What is the pathophysiology of vasculitis?
Inflammation of blood vessels can lead to ischaemia, tissue necrosis, and organ damage.
Often autoimmune
What systemic symptoms are common to all types of vasculitis?
Fever, malaise, weight loss, and fatigue
What factors determine the clinical presentation of vasculitis?
(1) The size and location of the affected vessels.
(2) The degree of organ dysfunction and inflammation
What is a key caution regarding ANCA testing in vasculitis?
ANCA is not a diagnostic test for all types of vasculitis
What are the potential consequences of poor management of vasculitis?
Permanent organ damage
Is vasculitis a single disease?
nope
What is the primary feature of large vessel vasculitis?
Chronic granulomatous inflammation predominantly affecting the aorta and its major branches
What arteries are affected in Takayasu arteritis?
Arteries branching from the aortic arch
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.
What are the early clinical features of Takayasu arteritis?
Non-specific symptoms like
(1) low-grade fever
(2) malaise
(3) night sweats
(4) weight loss
(5) arthralgia
(6) fatigue
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
What investigations are used for Takayasu arteritis?
Bloods: Raised inflammatory markers (ESR, CRP)
Imaging: MR/CT angiogram to detect thickened vessel walls and stenosis
What arteries are affected in Giant Cell Arteritis?
Carotid artery and its branches.
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.
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
What are the signs of giant cell arteritis?
(1) Tender, enlarged, non-pulsatile temporal arteries
(2) Carotid bruit
What is a key diagnostic clue for GCA in older adults?
A new-onset headache in patients aged >50 with elevated ESR or CRP
What is the gold standard for investigating giant cell arteries?
Temporal artery biopsy
What is the first-line treatment for both Takayasu arteritis and Giant Cell Arteritis?
Prednisolone 40–60 mg daily
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.
What is the long-term management strategy for Takayasu arteritis?
methotrexate or leflunomide
What is the emergency treatment for GCA with visual disturbances or complications?
High-dose intravenous steroids
Giant Cell Arteritis (GCA) can lead to what?
Ischaemic stroke
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
Confirmation of the diagnosis of giant cell arteries is what?
vascular ultrasound and temporal artery biopsy
When would low-dose Prednisolone, once daily be used instead of high dose?
12-18 months later
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: 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?
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
IV
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?
- Jaw claudication
- Headache
- Scalp tenderness
- Visual loss
What is the affected artery in Giant Cell Arteritis?
Temporal artery
What is the treatment of giant cell arteritis with visual symptoms?
High dose systemic corticosteroids
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
