23. ZTF vasculitis Flashcards
what is vasculitis
the name for inflammation of the blood vessels
they are characterised on weather they affect small, medium or large vessels
name the types of vasculitis that affect the small vessels
- Henoch-Schoenlein purpura
- Eosinophilic Granulomatosis with Polyangiitis (EGPA) (Churg-Strauss syndrome)
- Microscopic polyangiitis (MPA)
- Granulomatosis with polyangiitis (Wegener’s granulomatosis)
name the types of vasculitis that affect the medium sized vessels
- Polyarteritis nodosa
- Eosinophilic Granulomatosis with Polyangiitis (EGPA) (Churg-Strauss syndrome)
- Kawasaki Disease
name the types of vasculitis that affect the large vessels
- Giant cell arteritis (more likely in over 50s)
* Takayasu’s arteritis (more likely in under 50s)
What are the generic features/presentation that apply to most types of vasculitis
- Purpura. These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin.
- Joint and muscle pain
- Peripheral neuropathy
- Renal impairment
- Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding)
- Anterior uveitis and scleritis
- Hypertension
what markers are usually raised in vasculitis
inflammatory markers CRP and ESR
what are the two types of Anti-neutrophil cytoplasmic antibodies (ANCA)
p- ANCA aka anti-MPO antibodies
c- ANCA aka anti-PR3 antibodies
which type of vasculitis is associated with p-ANCA (MPO antibodies)
Microscopic polyangiitis
Churg-Strauss syndrome (aka Eosinophilic Granulomatosis with Polyangiitis (EGPA) )
which type of vasculitis is associated with c-ANCA (PR3 antibodies)
Wegener’s granulomatosis
give some examples of the different steroids that are used in the management of vasculitis
- Oral (i.e. prednisolone)
- Intravenous (i.e. hydrocortisone)
- Nasal sprays for nasal symptoms
- Inhaled for lung involves (e.g. Churg-Strauss syndrome)
give some examples of immunosuppressants that can be used to manage vasculitis
- Cyclophosphamide
- Methotrexate
- Azathioprine
- Rituximab and other monoclonal antibodies
which two of the vasculitis mainly affect children
henoch-schoenlein purpura (HSP) and Kawasaki
which antibody is involved in HS purpura
IgA vasculitis and so inflammation occurs due to immunoglobulin A deposits in the blood vessels of the affected organs such as the skin, kidneys and GI tract
what is henoch-schoenlein purpura (HSP) often triggered by
an upper airway infection eg tonsillitis or gastroenteritis
most common in children under 10
what are the four classic features of henoch-schoenlein purpura (HSP)
purpura (100%), joint pain (75%), abdominal pain (50%) and renal involvement (50%). HSP affects the kidneys in about 50% of patients, causing an IgA nephritis.
management is typically supportive
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome) most associated with lung and skin but can affect other organs such as kidney
how does it often present
with severe asthma in late teenage years or adulthood
A characteristic finding is elevated eosinophil levels on the full blood count.
Microscopic polyangiitis is a small vessel vasculitis. what is the main feature of microscopic polyangiitis
renal failure
can also affect the lungs causing SOB and haemoptysis
Granulomatosis with polyangiitis (Wegener’s granulomatosis)
it is a small vessel disease that affects the respiratory tract and kidneys. How does this classically present in exams
saddle shaped nose due to a perforated nasal septum which causes a dip halfway down the nose
other causes; epistaxis crusty nasal secretions hearing loss sinusitis
in the lungs;
cough, wheeze and haemoptysis
what can Granulomatosis with polyangiitis (Wegener’s granulomatosis) often be misdiagnosed as on a CXR
pneumonia
Polyarteritis nodosa (PAN) is a medium vessel vasculitis. what is it most associated with
hepatitis B but can also occur without a clear cause or with hepatitis C and HIV.
polyarterittis nodosa is associate with a rash known as
livedo reticularis. This is a mottled, purplish, lace like rash
Kawasaki disease is a medium vessel vasculitis that affects young children, typically under 5. There is no clear cause
what are the clinical features
- Persistent high fever > 5 days
- Erythematous rash
- Bilateral conjunctivitis
- Erythema and desquamation (skin peeling) of palms and soles
- “Strawberry tongue” (red tongue with prominent papillae)
what is the key complication with Kawasaki disease and the treatment
coronary artery aneurysm
aspirin and IV immunoglobulins
known as muco-cutaneous lymphadenopathy
Takayasu’s arteritis is a form of large vessel vasculitis
where does it mainly affect
the aorta and its branches
it also affects the pulmonary arteries
can swell and form aneurysms or become narrowed and blocked which leads to its other name of ‘pulseless disease’
how does Takayasu’s arteritis usually present
before the age of 40 years with non-specific systemic symptoms, such as fever, malaise and muscle aches, or with more specific symptoms of arm claudication or syncope.
It is diagnosed using CT or MRI angiography. Doppler ultrasound of the carotids can be useful in detecting carotid disease
