What is vasculitis?
Inflammation of blood vessels
What vessel changes would be expected in vasculitis?
- Vessel wall thickening
- Vessel wall stenosis
- Occlusion of vessels with subsequent infarction
What does the term "large vessel vasculitis" refer to?
Primary vasculitis causing granulomatous inflammation of the aorta and its major branches
What are the two major categories of large vessel vasculitis?
- Giant cell arteritis
- Takayasu arteritis
How does the age of onset differ in patients with giant cell arteritis vs Takayasu arteritis?
- Giant cell arteritis > 50 years
- Takayasu arteritis < 50 years
Where is Takayasu arterits most common in the world and which gender does it tend to affect?
East Asian countries
Females (young 20-30)
Giant cell arteritis typically affects which arteries?
Early features of large vessel vasculitis include which things?
- Low-grade fever
- Night sweats
- Weight loss
- Claudicant symptoms
If untreated, what can occur with large vessel vasculitis?
- Vascular stenosis
- Reduced pulses and bruit
Which imaging technique can detect thickened vessel walls and stenosis in large vessel vasculitis?
What is the rough dosage of steroids for
a) Large vessel vasculitis
b) Giant cell arteritis with ocular involvement
(1mg/Kg up to a max of 80mg)
As well as steroids, what other treatments can be added for large vessel vasculitis?
Methotrexate or azathioprine
Which small vessel vasculitides are ANCA associated?
- Granulomatosis with polyangiitis
- Microscopic polyangitiis
- Eosinophilic granulomatosis with polyangiitis
- Renal limited vasculitis
Which small vessel vasculitis is associated with ANCA, but not with granuloma?
Which small vessel vasculitis is ANCA associated, has granulomas present and is also associated with asthma and eosinophilia?
Eosinophilic granulomatosis with polyangiitis
Which non-ANCA associated small vessel vasculitis involves and IgA dominant immune deposit?
Which non-ANCA associated small vessel vasculitis has high levels of cryoglobulin?
Describe what granulomatosis with polyangiitis is
Granulomatous inflammation involving the respiratory tract combined with necrotising vasculitis of small and medium sized vessels.
Necrotising glomerlulonephritis is common
Describe what microscopic polyangiitis is
Necrotising vasculitis affecting small vessels
Both necrotising glomerulonephritis and pulmonary capillaritis are common
Describe what renal limitied vasculitis (Pauci-immune) is
There is Pauci-immune vasculitis (little evidence for hypersensitivity when staining for IgG) and no apparent extra-renal features on presentation.
There is only necrotising glomerulonephritis present
Describe what eosinophilic granulomatosis with polyangiitis is
Granulomatous inflammation in the respiratory tract
Necrotising vasculitis affecting small and medium sized vessels is present
The condition is associated with eosinophilia and asthma
Which types of symptoms are common in granulomatosis with polyangiitis?
- Nose bleeds
- Recurrent sinusitis
- Nasal crusting
- Saddle nose damage
There may also be haemoptysis and cavitating lesions on CXR
Rhinitis is often the first sign
GPA is associated with ______ and ____
cANCA and PR3 (proteinase 3)
How is EGPA characterised?
Late onset asthma, rhinitis and raised peripheral blood eosinophil count
Whcih other symptoms besides the characteristic symptoms of EGPA are also common?
EGPA is associated with ____ and ___
pANCA and MPO (myeloperoxidase)
What is the most important complication of microscopic polyangiitis?
(up to 90% of patients experience)
Which investigations are appropriate for vasculitis?
- ESR, PV and CRP (raised)
- FBC (anaemia is common)
- U+E (renal involvement)
- Urinalysis (renal vasculitis)
- Biopsy of affected area
How are most cases of ANCA associated vasculitis treated?
- IV steroids
What is Henoch-Schonlein purpura?
An acute IgA-mediated disorder characterised by a generalised multi-system small vessel vasculitis
Which areas of the body does Henoch-Scholein purpura typically affect?
- GI tract
- Lungs (More rare)
- CNS (more rare)
Henoch-Scholein purpura typically affect what age group of people?
Henoch-Scholein purpura is usually preceeded by a history of what?
Upper respiratory tract infection
Common symptoms of Henoch-Scholein purpura include what?
- Purpuric rash over buttocks and lower limbs
- Abdominal pain
- Joint pain
What is the treatment for Henoch-Scholein purpura?
It is genrally a self-limiting condition which doesn't require specific treatment
It will usually settle over the course of weeks and months