Vasculitis Flashcards
What is vasculitis?
Inflammation of blood vessels
What can the inflammation in vasculitis result in?
Vessel wall thickening, stenosis and occlusion with subsequent ischaemia, necrosis and organ inflammation
What blood vessels can vasculitis affect?
Any (i.e. artery, vein, capillary)
What system does vasculitis affect?
It is a multi-system disease
What is the mortality rate of small vessel vasculitis if left untreated for 2 years?
90%
What is primary vasculitis?
Results from an inflammatory response that targets the vessel walls and has no known cause (can be autoimmune)
What is secondary vasculitis?
May be triggered by an infection, drug or toxin or as part of another inflammatory disorder or cancer
What cell mediates vasculitis?
T cells
How are small vessel vasculitis’ split into two categories?
ANCA positive ANCA negative
If there is ANCA + vasculitis, and granulomas are present, what could this be?
EGPA or GPA
If there is ANCA + vasculitis and there are no granulomas present, what would this be?
MPA
What are some systemic features of vasculitis which may be present in all types?
- Fever - Malaise - Weight loss - Fatigue - Night sweats - Arthralgia
What is large vessel vasculitis?
Primary vasculitis which causes chronic granulomatous inflammation, predominantly of the aorta and its major branches
What are the two types of large vessel vasculitis?
- Takayusu Arteritis - Giant Cell (Temporal) Arteritis
Which arteries are often affected in Takayusu Arteritis?
Aorta, femoral and subclavian arteries
Who does Takayusu Arteritis normally affect?
Those < 50, predominantly women in their 2nd-3rd decade
Which population is Takayusu Arteritis most common in?
Asians
Who does Giant Cell Arteritis usually affect?
> 50s
What arteries are affected by Giant Cell Arteritis?
Usually the temporal artery, but the aorta and other large vessels can be involved
What can occur if large vessel vasculitis is left untreated?
Vascular stenosis and aneurysms which can cause reduced pulses and bruits
What are 5 presenting features of large vessel vasculitis?
- Bruit - BP difference in extremities - Claudication - Carotodynia or vessel tenderness - Hypertension
Where is a bruit most commonly found in large vessel vasculitis?
Carotid arteries
What disease does temporal arteritis have an association with?
Polymyalgia rheumatica
What is the major risk of temporal arteritis?
Blindness due to ischaemia of the optic nerve
What are some investigations for large vessel vasculitis? What will they show?
- Inflammatory markers will be raised - MR angiogram (may show thickening, stenosis or aneurysm) - PET CT (shows increased metabolic activity of inflamed vessels)
What investigation is done if there is suspected temporal arteritis? Is this always sensitive?
Temporal artery biopsy- there are skip lesions so can show up negative even when the condition is present
What is the treatment for large vessel vasculitis?
40-60mg prednisolone, gradually reducing dose Can use steroid sparing agents such as azathioprine or methotrexate
What are the two types of medium vessel vasculitis?
Kawasaki Disease Polyarteritis Nodosa
Who is Kawasaki Disease seen in?
Children, mainly < 5 years
Which vessels does Kawasaki Disease affect?
Various ones, the most important being the coronary arteries where aneurysms can develop
What characterises Polyarteritis Nodosa?
Necrotising inflammatory lesions that affect arteries at vessel bifurcations, resulting in aneurysms
Which sites does Polyarteritis Nodosa affect?
Skin, gut, kidneys
Polyarteritis Nodosa is associated with what other disease?
Hepatitis B
Describe the pathology of GPA (Wegener’s)?
Granulomatous inflammation of the respiratory tract, small and medium vessels. Necrotising glomerulonephritis is common.
Describe the pathology of EGPA (Churg-Strauss)?
Eosinophilic granulomatous inflammation of the respiratory tract, small and medium vessels. Associated with late onset asthma.
Describe the pathology of MPA?
Necrotising vasculitis with few immune deposits. Necrotising glomerulonephritis is very common.
Where does MPA only really affect?
The kidneys
GPA is more common in individuals from where?
North Europe
What sex is GPA more common in?
Males, but not by much
What is the age onset of GPA?
Can occur at any age, but typically 35-55 years
A person has GPA if two of what four features are present?
- Nasal or oral inflammation - Abnormal chest radiograph - Urinary sediment - Granulomatous inflammation on biopsy
What are some ENT features of GPA?
Sinusitis, nasal crusting, epistaxis, mouth ulcers, deafness, saddle nose
What causes saddle nose in GPA?
Cartilage ischaemia
What are some respiratory features of ANCA vasculitis?
Pulmonary infiltrates, cough, haemoptysis, nodules on x-ray
What are some cutaneous features of ANCA vasculitis?
Non-blanching purpura, cutaneous ulcers
What does necrotising glomerulonephritis manifest as?
Blood and protein in the urine
What are some neurological features of ANCA vasculitis?
Mononeuritis multiplex, polynephropathies, cranial nerve palsies
What is mononeuritis multiplex? Which ANCA vasculitis is it more common in?
A peripheral nerve stops working because of a lack of blood flow- more common in EGPA
What are some ocular features of ANCA vasculitis?
Conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, retinal artery occlusion, proptosis
What is the main difference in the presentation of EGPA compared with GPA?
Late onset asthma, high eosinophil count, rhinitis
Eosinophilia must be of what percent in the blood to diagnose EGPA?
> 10%
What is ANCA?
Antibodies against antigens in the cytoplasm of neutrophil granulocytes
Does every patient with GPA, EGPA, MPA have ANCA antibodies?
No
What test is used to detect ANCA?
Immunofluorescence
cANCA and PR3 suggest what disease?
GPA
pANCA and anti-MPO suggest what disease?
EGPA
Which is more specific, cANCA or pANCA?
cANCA
Why can cANCA, pANCA, PR3 and anti-MPO be useful monitoring tools?
They vary with disease activity
ANCA associated vasculitis is associated with the formation of what?
Immune complexes
What happens to complement levels during disease activity?
Decreased
What is the most common treatment for ANCA associated vasculitis, especially if there is evidence of renal disease?
IV steroids and cyclophosphamide
What would be the treatment for localised ANCA vasculitis with no systemic involevement, or early systemic involvement?
Methotrexate/azathioprine + steroids
If cyclophosphamide and steroids do not work as a treatment for ANCA vasculitis, what is the next step?
Rituximab and steroids
What treatment is used in ANCA vasculitis if creatinine is > 500?
Plasma exchange
What is used for ANCA vasculitis treatment for very progressive disease, unresponsive to all other treatments?
IV immunoglobulins and rituximab
What investigations are used for ANCA vasculitis?
- Inflammatory markers (raised) - Anaemia - U+Es and urinalysis to look for renal involvement - ANCA - CXR - Biopsy of affected area
What is an example of a non-ANCA small vessel vasculitis?
Henoch Schonlein purpura
What mediates Henoch Schonlein purpura?
IgA
Who does Henoch Schonlein purpura usually occur in?
Children
What triggers Henoch Schonlein purpura?
A preceding infection, usually URTI caused by group A strep, though can be a GI infection
How long does the preceding illness usually predate the onset of Henoch Schonlein purpura?
1-3 weeks
How does Henoch Schonlein purpura present?
A purpuric rash over the buttocks and lower limbs, can also have colicky abdominal pain, joint pain, bloody diarrhoea, renal involvement
How long does Henoch Schonlein purpura last for?
This is a self-limiting condition which usually resolves in 8 weeks but can relapse
What should you always do when someone presents with Henoch Schonlein purpura?
Urinalysis for renal involvement
What type of ANCA is this?
cANCA
What type of ANCA is this?
pANCA
