Vasculitis Flashcards
(44 cards)
What is vasculitis?
Inflammation of the blood vessels. Often with ischaemia, necrosis and organ involvement.
Which blood vessels does vasculitis affect?
Can affect any of them.
Why is diagnosing vasculitis difficult?
The clinical manifestations are diverse and can affect any organ/system. Therefore pin pointing this is tricky.
Primary vasculitis
Results from an inflammatory response that targets the vessel walls- no known cause.
Sometimes this can be autoimmune.
Secondary vasculitis
May be triggered by an infection, a drug, a toxin or may occur as part of another inflammatory disorder or cancer.
Pathogenesis of vasculitis
Activated dendritic cells release inflammatory mediators that promote the activation of T cells and cause inflammation, granuloma formation and macrophage activation.
Activated macrophages cause progressive endothelial damage, disruption of the internal elastic lamina and intimal hyperplasia.
Classification of vasculitis
International Chapel Hill consensus classification system
Could be large vessel e.g. Giant cell arteritis and Takayasu
Could be medium vessel e.g. Polyarteritis nodosa and Kawasaki disease
Or small vessel which are made up of immune complex associated vasculitis or ANCA associated vasculitis.
Large vessel vasculitis
Primary vasculitis causing chronic granulomatous formation primarily affecting the aorta and its major branches.
Categories of large cell vasculitis
Giant cell arteritis and takayasu arteritis.
Takayasu arteritis aetiology
Generally affects people of less than 40 years old and is much commoner in females. More common in Asian populations.
Giant cell arteritis aetiology
Generally affects those over 50.
Similarities between Takayasu arteritis and giant cell arteritis
They are both characterised by granulomatous infiltration in the walls of large vessels.
Presenting features of large cell vasculitis
Bruit- most commonly in the carotid artery
Blood pressure difference in the extremities
Claudication
Vessel tenderness or carotodynia (unilateral tenderness of the carotid artery)
Hypertension
Temporal arteritis association with other diseases
Associated strongly with polymyalgia rheumatica. About 50% of people with GCA have PMR.
Temporal arteritis symptoms
Unilateral headache Scalp tenderness Jaw claudication Temporal arteries may be prominent with reduced pulsation. Risk of blindness.
Investigations into large cell vasculitis
Inflammatory markers (ESR, CRP, Plasma viscocity) usually raised.
If there are symptoms of temporal arteritis- then temporal artery biopsy is needed.
Imaging such as MRI and pet scan may show vessel wall thickening/stenosis/aneurysm
Treatment of large cell vasculitis
Steroids- 40-60mg of prednisolone
Steroid sparing agents such as methotrexate could be considered.
Kawasaki disease aetiology
Seen in children- usually under 5 years.
Where does Kawasaki’s disease affect?
Can affect many vessels but predominantly the coronary arteries. This is where aneurysms can develop.
What is polyarteritis nodosa
Necrotising inflammatory lesions that arteries at vessel bifurcations resulting in microaneurysmal formations and aneurysm formations.
What is polyarteritis nodosa associated with?
Hepatitis B. It can also affect the skin, gut and kidneys.
Divisions in small vessel vasculitis
ANCA positive and ANCA negative.
New name for Wegners granulomatosis
Granulomatosis with polyangitis
New name for Churg Strauss syndrome
Eosinophillic granulomatosis with polyangitis
