Vasculitis Flashcards
What occurs in large vessel vasculitis?
Chronic granulomatous inflammation usually of the aorta and main branches.
Two main types of large vessel vasculitis
Takayasu under 50 years
Giant Cell arteritis over 50 years
Takayasu
Under 50 year old East Asian female.
Claudication and bruits usually in the carotids.
Blood pressure differences in extremities
Giant Cell Arteritis
Over 50
Temporal artery involvement is common but can also affect other large arteries.
If untreated what can occur in large vessel vasculitis.
Vascular stenosis and distal ischaemia and aneurysm.
What investigations are used in large vessel vasculitis?
FBC- raised CRP and plasma viscosity
MR angiography- shows stenosis and thickened walls
Temporal artery biopsy - GCA
PET scan- shows increased metabolic activity in large vessels.
Treatments for large vessel vasculitis?
40-60mg of prednisolone.
Steroid sparing methotrexate may be used.
What is the primary medium vessel vasculitis and who does it usually affect?
Kawasaki
Usually children under 5
What are the to main types of small vessel vasculitis?
ANCA +ve
ANCA -ve
How are all small vessel vasculitis presentations similar?
Fever and weight loss Raised non blanching purpuric rash Arthralgia Glomerulonephritis Lung opacities on x-ray
Name 4 types of ANCA positive vasculitis
Granulomatosis with polyangitis
Microscopic Polyangitis
Renal Limited vasculitis
Eosinophilc granulomatosis with polyangitis
What is Granulomatosis with polyangitis also known as?
Wegeners
How does GPA usually present?
Sinusitis Mouth Ulcers Saddle Nose Otitis media Deafness Uveitis and conjunctivitis
GPA with respiratory involvement
Haemoptysis
Pulmonary infiltrates and cavitating nodules on X-ray
GPA with cutaneous involvement
palpable purpura
and ulceration
GPA with renal involvement
Necrotising glomerulonephritis
GPA with CNS involvement
Nerve palsy
What is GPA associated with?
cANCA and PR3 proteinase 3
Is GPA more common in men or women?
M:F 1.5:1
Over 35
90% N.Europe
How is eosiniphilc granulmatoses with polyangitis different to GPA
Presents the same but Late onset Asthma
Raised blood eosinophil count
Rhinitits
What is different immunological between EGPA and GPA
EGPA is pANCA
GPA in cANCA
Low complement levels in both
What antibodies are associated with EGPA
pANCA
Anti NPO
Small Vessel Vasculitis Investigations
FBC- PV and CRP raised
Anaemie- common in chronic
Immunology- ANCA Biopsy
Imaging- Chest Xray
Investigations if suspected renal involvement?
Urine dipstick for proteinuria
Urgent kidney biopsy
Management of ANCA +ve disease
Localised/Early
- Methotrexate
Systemic/Agresive/Late
- Cyclophosphamide and steroid
- Rituximab and steroid
Followed by azathioprine or methotrexate once controlled.What is used in refractory ANCA +ve disease?
IV immunoglobulins and rituximab
Henoch Schnlien Purpura
ANCA -ve disease
Ig-A dominant immune deposits.
Who is usually affected by HSP?
75% children with a preceding infection Upper respiratory tract infection around 2-3 weeks prior.
What group of bacteria are commonly linked to HSP
Group A Streptococcus
What is HSP
Generalized small vessel vasculitis affecting the: Skin GI Tract Kidneys Joints.
Where does HSP rarely affect?
The lungs
How does HSP present?
Purpuric rash over buttocks and lower limbs.
Abdominal pain
D+v
Arthralgia
Does HSP require treatment?
Its usually self limiting treatment is usually supportive.
