Vasculitis Flashcards
Define vasculitis?
Vasculitis is an inflammatory disorder of blood vessel walls, causing destruction (aneurysm/rupture) or stenosis.
Can affect vessels of any organ and presentation depends on which organs are involved
Can be primary or secondary to other diseases e.g. SLE, RA, hep B and C, HIV
what are the 3 types of large vessel vasculitis?
giant cell arteritis
polymylagia rheumatica
takayasu’s arteritis
what is the target artery, key info and classic presentation of giant cell arteritis?
target artery- carotid artery and branches
key info
- affects elderly
- associated with PMR
- Treat urgently with oral predinsiolne
Classic presentation
- Temporal artery- headache, scalp tenderness
- Facial artery- jaw claudication
- opthalmic artery- loss of vision
what is the target artery, key info and classic presentation of polymylagia rheumatica?
target artery- unknown
key info
- associated with GCA
- treat with oral steroids
Classic presentation
- bilateral pain in morning
- stiffness in pelvic, neck and shoulder girdle muscles
- no weakness
what is the target artery, key info and classic presentation of takayasu’s arteritis?
target artery- branches of aortic arch
key info- affects asian women ( 20-40 yrs)
classic presentation
- inflammatory phase: FLAWS
- pulseless phase: weak/absent UL/LL pulses, claudication
state 2 medium vessel vasculitis?
polyarteritis nodosa
kawasaki
what is the target artery, key info and classic presentation of polyarteritis nodosa?
target artery: skin, renal and mesenteric
key info
- associated with Hep B
- Rosary sign on renal angiogram
classic presentation
- consitiutional upset
- skin rash
- abdominal pain
- GI bleed
- hypertension
- renal failure
- peripheral neuropathy
what are the two divisions of small vessel vasculitis?
ANCA positive and ANCA negative
what are the 3 types of ANCA positive vasculitis?
granulomatosis with polyangitis ( Wegner’s)
Eosinophilic granulomatosis with polyangitis
microscopic polyangitis
what is the ANCA, key info and classic presentation of granulmatosis with polyangitis ( Wegener’s)?
cANCA
Key info
- Saddle node
- can cause cavitating lung lesions
Classic presentation
- upper resp tract: nosebleeds, rhinitis , sinusitis
- lower resp tract: haemoptysis
- kidneys: glomerulonephritis
what is the ANCA, and classic presentation of microscopic polyangitis?
pANCA
Rapidly progressing glomeulonephritis
pulmonary haemorrhage ( haemoptysis)
what is the ANCA, key info and classic presentation of eosinophilic granulomatosis with polyangitis?
pANCA
Triphasic
allergic phase- asthma/ rhinitis
eosinophillic phase- high eosinophills-> tissue damage
vasculitic phase- widespread organ damage and death
what are the 3 types of ANCA negative small vessel vasculitis?
Hennoch schonlein purpura
Bechets disease
Goodpasture’s syndrome
What are the key features and classic features of Hennoch Schonlein purpura?
key info
- affects 3-15 yr old children
- IgA vasculitis
Classic presentation- TRIAD
- purpura rash on buttocks and extensors of lower limbs
- abdominal pain
- arthralgia of larger joints
What are the key features and classic presentaiton of Bechet’s disease?
key info
- more common in Greek and turkish people
classic presentation- TRIAD
- recurrent oral ulcers
- genital ulcers
- uveitis
what are the key features and classic presentation of Goodpasture’s syndrome?
key info
- anti-GBM antibodies
Classic presentation- TRIAD
- glomerulonephritis-> renal failure
- haemotysis ( Pulmonary haemorrhage
- anti- GBM anitbodies
what are the risk factors for vasculitides?
Hepatitis B- polyarteritis nodosa
Hepatitis C - mixed essential cryoglobulinaemia
pANCA- microscopic polyangiitis + Churg-Strauss ( eosinophilic granulomatosis with polyangitis)
c-ANCA- Wegner’s granulomatosis
Hx of asthma, allergic rhinitis or sinusitis – Churg-Strauss
outline the aetiology of vasculitides?
Suggested autoimmuneorigin
WBCs mistake self-antigens on endothelial cells as foreign antigens due to molecular mimicry and hence cause damage.
Immune complex deposition in the walls of blood vessels leads to inflammation
In other cases, WBCs damage cells near the endothelial cells and as a result, endothelial cells get damagedindirectly. This tends to occur in the small vessels.
The vessel wall damage leads to three things:
- The damage exposes underlying collagen and TF increasing coagulation
- The weaker walls also make aneurysmsmore likely
- Healing of the walls causesscarring fibrosis which makes the vessels stiffer and narrower
=> reduced blood flow to organs downstream causing ischaemia
summarise the epidemiology of vasculitides?
RARE
Takayasu’s arteritisis most common in JAPANESE FEMALESand women < 40
GCA is most common in women over 55
Granulomatosis with polyangiitis most common in middle aged men
Kawasaki most common in children <5, especially boys
what is the general rule for the signs and symptoms seen in vasculitides?
Large vessel vasculitides have classic clinical patterns based on the vessels affected (e.g. GCA and loss of vision/headache)
Medium and small vessel vasculitides are characterised by multiorgan involvement and have less specific clinical features
what are the features of GCA?
loss of vision, jaw claudication, headache, scalp tenderness
What are the features of Takasayu’s?
Takayasu: affects arteries branching off from the aortic arch.
If it affects parts supplying upper extremities, you get weak or no pulse.
If it affects supplying the head, you get visual and neurological symptoms
what are the feartures of kawasaki?
Affects coronary arteries
Can cause MI
Causes conjunctivitis, rash, lymphadenopathy, strawberry tongue, swollen hands and feet, and fever
what are the features of polyarthritis nodosa?
This occurs when immune cells directly damage endothelium confusing it with hep B cells
Association with hepatitis B
Causes transmural inflammation of entire blood vessel wall, which leads to necrosis and then fibrosis. The weak fibrosis leads to fibrotic aneurysmsalong the vessel wall
Can occur anywhere causing organ ischaemia
If it affects renal arteries, it causes hypertension
If it affects mesenteric arteries, causes mesenteric ischaemia and severe abdo pain
Signs: typical systemic features, skin rash and punched out ulcers, microaneurysms, thrombosis, infarctions, hypertension, testicular pain
describe the aetiology of ANCA positive small vessel vasculitis?
For ANCA positive diseases, B cells mistakenly target their antibodies (called ANCA) against granules made by neutrophils
This causes neutrophils to release oxygen free radicals that cause indirect damage to nearby endothelial cells
ANCAs are usually IgG and there are two types: cANCA and pANCA
what are the features of granulomatois with polyangitis ( Wegener’s)
- Affects nasopharynx, lungs and kidneys
- Nasopharynx: chronic pain from sinusitis, bloody mucous and nasal discharge, saddle nose shape
- Lungs: breathing difficulties, haemoptysis, chest pain, etc
- Kidneys: glomerulonephritis, decreased urine production, hypertension
- cANCA
what are the features of microscopic polyangitis?
Very similar to Wegener’s but does not affect nasopharynx, only kidneys and lungs
Also pANCAassociated rather than cANCA
what are the features of Eosinophilic granulomatosis with polyangiitis (Churgg-Straus syndrome)
- Associated with pANCAs
- Affects nasopharynx, kidneys, lungs, GI, skin, nerve and heart
- Nasal discharge or stuffiness – purulent or bloody with facial pain
- Associated with asthma(so WHEEZE), allergic rhinitis or sinusitis
- Has high eosinophil count
what are the features of Henoch- schonlein purpura?
- This is not ANCA associated, but rather due to high IgA antibodies in the blood which directly target endothelial cells due to molecular mimicry
- Purpura, arthritis, gut symptoms (abdominal pain), glomerulonephritis (haematuria), IgA deposition
- The purpura is palpable – can feel it raised above skin due to fibrosis

what are the investigations for vasculitides?
Bloods
Autoantibodies - e.g. cANCA in Wegner’s
HBV serology – for polyarteritis nodosa
Urine - haematuria, proteinuria, red cell casts (if glomerulonephritis)
Microscopic haematuria is common for Wegner’s
PFTs – check for asthma esp for Churg-stauss
CXR - diffuse, nodular or flitting shadows, atelectasis
Biopsy - renal, lung, temporal artery – in GCA, but note that GCA is segmental so biopsy requires a long section
Echo – to see if there is pericardium involvement
Angiography - to identify aneurysms
interpret the results in vasculitides?
FBC - normocytic anaemia, high platelets, high neutrophils
Eosinophil count – increased with Churg-strauss
High ESR/CRP
Urea and Creatinine high for renal failure
Complement – may be low in polyarteritis nodosa
LFTs – mild elevation is common esp in polyarteritis nodosa