vasculitis Flashcards
what can vasculitis lead to
inflammation
ischemia
necrosis
what causes primary vasculitis
an inflammatory response that targets the vessel walls and has no known cause
what causes secondary vasculitis
may be triggered by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder or cancer
clinical features of vasculitis
depends on which vessel is affected
- fever
- malaise
- weight loss
- fatigue
what are the categories of vasculitis
- medium vessel vasculitis
- immune complex small-vessel vasculitis
- ANCA-associated small-vessel vasculitis
- large vessel vasculitis
what are the two main causes of large cell vasculitis
- takayasu arteritis
- Giant cell arteritis
pathophysiology of vasculitis
granulomatous infiltration of the walls of the large vessels
features of Takayasus arteritis
- under 40
- females
- claudication
- asian populations
- bruit
- carotid artery
- bp difference of extremities
- angiogram
features of giant cell arteritis
- over 50
- temporal arteritis
- headache
- scalp tenderness
- visual disturbances
- jaw claudication
- associated with polymyalgia rheumatica
investigations for giant cell arteritis
- ESR or plasma viscosity
- CRP raised
- temporal artery biopsy
- USS
- PET CT or CT angiogram
management for large vessel vasculitis
- start on 40-60mg prednisolone
- steroid sparing agent may be considered
- Tocilizumab
what is the pathophysiology of granulomatosis with polyangitis
Granulomatous inflammation of respiratory tract, small and medium vessels. Necrotising glomerulonephritis common.
what is the pathophysiology of eosinophilic granulomatosis with polyangitis
Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels. Associated with asthma
what is the pathophysiology of microscopic polyangitis
Necrotising vasculitis with few immune deposits. Necrotising glomerulonephritis very common
what are the ENT features of GPA
- sinusitis
- nasal crusting
- epistaxis
- mouth ulcers
- sensorineural deafness
- otitis media and deafness
- saddle nose
- sub glottic inflammation
ocular features of GPA
- conjunctivitis
- episcleritis
- uveitis
- optic nerve vasculitis
- retinal artery occlusion
- proptosis
resp features of GPA
- cough
- hemoptysis
- pulmonary infiltrates
- diffuse alveolar haemorrhage
- cavitating nodules on CXR
cutaneous features of GPA
palpable purpura
cutaneous ulcers
renal features of GPA
necrotising glomerulonephritis
nervous system features of GPA
- mononeuritis multiplex
- sensorimotor polyneuropathy
- cranial nerve palsies
what the main differences between EGPA and GPA
EPGA has late onset asthma, a high eosinophil count and ANCA
what is Henoch-Schonlein Purpura
it is an acute immunoglobin A mediated disorder
-generalized vasculitis involving small vessels of the skin, GI tract, kidneys, joints and rarely lungs and CNS
when do most cases of HSP occur
2-11 yrs
most also have a preceding URTI, pharyngeal infection or GI infection
presentation of HSP
- purpuric rash - buttocks and limbs
- colicky abdo pain
- bloody diarrhoea
- joint pain +/- swelling
- renal involvement
investigations for vasculitis
- urine dipstick
- FBC, inflammatory markers
- ANCA and specific antibodies
- connective tissue disease screen
- compliment levels
- imaging
- nerve conduction tests
- tissue biopsy
is cANCA associated with GPA or EPGA
GPA
is pANCA associated with GPA or EPGA or MPA
EPGA and MPA
what is it essential to do in HSP
perform urinalysis to screen for renal involvement
