What is vasculitis?
Inflammation of blood vessels
How can vasculitis be classified? (2)
- Size of vessel
- ANCA positive or negative
What are the two major categories of large vessel vasculitis?
- Giant cell arteritis
- Takayasu arteritis
Which arteries are most common for giant cell arteritis to affect?
- Temporal artery (most common)
- Opthalmic artery
- Facial artery
The endothelium is generally directly attacked with molecular mimicry in which two classes of vasculitis?
- Large vessel
- Medium vessel
The endothelium is generally indirectly attacked in which class of vasculitis?
(N.b Henloch-Scholein purpura is an exeption to this rule)
What happens to the vessel walls due to the inflammation is vasculitis?
- Inflammatory processes cause tissue factor and collagen to be exposed (endothelial cells die)
- Thrombosis occurs
- Vessel walls weak leading to aneurysm
- Fibrosis due to fibrin deposition occurs as part of the healing process
- Both thrombosis and fibrosis narrow the vessel lumen reducing blood flow
Ultimately, vasculitis leads to less ______ _____
Why does jaw claudication occur with giant cell arteritis?
The facial artery is affected and less blood flow to the face occurs
Giant cell arteritis is most common in what age group and sex?
Women > 50
What is found in the elastic lamina in a patient with giant cell arteritis?
Granulomas or "giant cells"
When taking a biopsy for giant cell arteritis, why is a negative result not able to rule out the condition?
Giant cell arteritis is segmental
Takayasu arteritis is most common in which age group and sex?
Asian women < 40
Takayasu arteritis generally affects which arteries?
Those off of the aortic arch
In patients with Takayasu arteritis, what is found in the internal elastic lamina?
Granulomas or "giant cells"
What are some common medium vessel arteritides?
- Kawasaki disease
- Polyarteritis nodosa
Kawasaki disease generally affects people of what age?
(boys more than girls)
What are the symptoms of Kawasaki disease?
Conjuctivitis (sparing the limbus)
Rash over body
Adenopathy (large or swollen lymph nodes)
Hands and feet swelling
(Patients crash and burn)
Kawasaki disease is usually _____ __________
How can Kawasaki disease be treated?
Aspirin (monitor very closely for Reye syndrome)
What is the pathogenesis behind polyarteritis nodosa?
Immune cells directly attack the endothelial cells
This is an example of molecular mimicry as the antibodies produced are designed to takle hepatitis B
Polyarteritis nodosa causes ___________ necrosis
Fibrinoid necrosis occurs with polyarteritis nodosa which leaves the vessels prone to what?
Upon angiogram, polyarteritis nodosa has a very distinctive appearance. What is this appearance?
"String of beads"
In polyarteritis nodosa what happens when renal arteries are affected?
(low glomerular pressure leads to fluid retention and increased blood pressure)
What can occur when the mesenteric arteries are affected in polyarteritis nodosa?
- Abdominal pain
- GI bleed
What is Buerger's disease?
A medium vessle vasculitis affecting vessels in the fingers and toes
Blood clots occur as a result leading to ulceration, necrosis and auto-amputation of the fingers and toes
What is a major risk factor and potential trigger for Buerger's disease
Use of tobacco
What is ANCA?
Anti-neutrophil cytoplasmic antibody
Wegener's granulomatosis (Granulomatosis with polyangiitis) involves which type of ANCA?
In Wegener's granulomatosis, cANCA binds to what?
Proteinase 3 within the neutrophilic membrane
When pANCA or cANCA binds to neutrophils, why does the endothelium end up damaged?
O2 free radicals are released from neutrophils causing indirect damage
Name some small vessel vasculitides?
- Wegener's granulomatosis
- Microscopic polyangiitis
- Churg-Strauss syndrome
- Henloch-Schonlein purpura
- Renal limited vasculitis
Wegener's granulomatosis is more common in which sex?
Which areas of the body does Wegener's granulomatosis commonly affect?
Nasopharynx (painful sinusitis, bloody mucous from ulcers and saddle nose deformity)
Lungs (difficulty breathign due to ulcer formation and bloody cough)
Saddle nose deformity is associated with which vasculitis?
Once cured, relapse of Wegener's granulomatosis is what?
How is microscopic polyangiitis different from Wegener's granulomatosis?
- Does not affect nasopharynx (only kidneys and lungs)
- No granulomas can be found in vessle walls
- pANCA vs cANCA
What is pANCA?
After microscopic polyangiitis is cured or overcome, relapse is what?
Churg-Strauss syndrome is commonly mistaken for what?
Asthma or allergies
Which immune cell type is particularly elevated in Churg-Strauss syndrome?
Which areas of the body does Churg-Strauss syndrome affect?
- GI tract
Are granulomas associated with Churg-Strauss syndrome?
What makes Henloch-Schonlein purpura different from other small vessel vasculitides?
There are no ANCAs involved
What causes Henloch-Schonlein purpura?
IgA targets endothelial cells via molecular mimicry
The purpura seen in Henloch-Schonlein purpura is distinctive, but why?
It is palpable
This is due to the fibrosis occurng in the affected vessels
Henloch-Schonlein purpura can affect two other major body systems besides the skin, what are these?
GI (abdominal pain)
Renal (haematuria, IgA nephropathy)
Henloch-Schonlein purpura can resolve on its own.
True or false?
Large vessel vasculitis can lead to reduced _______ and carotid ________
What is the treatment for large vessle vasculitis?
40-60mg of prednisolone which granually reduces
What is the most common complication of microscopic polyangiitis?
Most cases of ANCA +ve vasculitis require what treatment?
Henloch-Schonlein purpura commonly affects children and a history involving what is common?
An upper respiratory tract infection predating symptom onset by a few weeks
Henloch-Schonlein purpura has what symptoms commonly?
- Purpuric rash over buttocks and lower limbs
- Abdominal pain
- Joint pain
Henloch-Schonlein purpura requires what treatment?
It is a self limiting condition which does nto require much specific treatment
It will settle over a period of weeks to months
Temporal arteritis has a common association with what?
cANCA and anti-PR3 are asscoiated with what?
pANCA and anti-MPO are associated with what?
Anti-MPO or anti-PR3 are asscoiated with what?
(Eosinophilic granulomatosis with polyangiitis)