VASCULITIS Flashcards
What happens in the vessel during vasculitis?
Dendritic cells –> cytokine release –> T cell activation –> Macrophage activation –> Progressive inflammatory response, endothelial damage –> Intima hyperplasia
What can be some systemic symptoms of vasculitis?
Fever, malaise, weight loss and fatigue
What are the two large vessel vasculitis?
Giant cell arteritis (Over 50, temporal), Takayasu arteritis (Under 40, females, Asains)
What are the presenting features of large vessel vasculitis?
Bruit, blood pressure difference of extremities, claudication, Carotodynia or vessel tenderness, hypertension
What condition is polymyalgia rheumatica associated with?
Temporal arteritis - GCA
What are the presenting symptoms?
Scalp tenderness, unilateral temporal headache, jaw claudication - risk of blindness, ischaemia of the optic nerve
Is there a specific blood test for temporal arteritis?
Nope, there is no specific antibody involvement
What age group is affected by Kawasaki disease? Where does it affect?
Under 5s
Can affect any vessels but affects the coronary arteries - can cause aneurysm
What can polyarteritis nodosa cause?
Lesions at vessel bifurcations, micro aneurysm formation
What are medium vessel vasculitis associated with?
Hep B
What is the new name for Wegener’s syndrome?
Granulomatosis with polyangitis - Granulomatous inflammation of respiratory tract, small and medium vessels. Necrotising glomerulonephritis common.
What is the new name for Churg Strauss syndrome?
Eosinophilic granulomatosis with polyangiitis (EGPA)
- Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels. Associated with asthma
What is the new name for Microscopic polyangiitis?
Trick question! Its not changed!
Affects kidneys, not so much lungs – doesn’t have the multisystem presentation
Necrotising vasculitis with few immune deposits. Necrotising glomerulonephritis very common
What are the symptoms and signs of granulomatosis with polyangitis?
- Nasal or oral inflammation, Chest nodules or cavities (cough, haemoptysis), microhematuria
- Sinusitis, nasal crusting, epistaxis, sensorineural deafness, otitis media, Saddle nose (cartilage ischaemia), palpable purpura (non blanching cutaneous lesions)
- Necrotising glomerulonephritis – blood and protein in urine
- Mononeuritis multiplex, cranial nerve palsy (would usually cause double vision)
- Conjunctivitis, uveitis
What is the main difference between GPA and EGPA?
The late onset of asthma and the high eosinophil count
What does Anti PR3 go along with?
cANCA - GPA
What does Anti MPO go along with?
pANCA - MPA and EGPA
How would you manage localised/ early systemic AAV?
Methotrexate and steroids
How would you manage generalised systemic AAV?
Cyclophosphamide and steroids
What may be a risk factor of AAV?
Nasal carriage of S.aureus
How would you describe Henoch - Schonlein Purpura
Acute generalised IgA mediated disorder - generalised vasculitis
What is Henoch - Schonlein purpura presentation?
- Purpuric rash over bum and lower limbs, colicky abdominal pain, bloody diarrhoea, joint pain and swelling and renal involvement
