Vasculitis Flashcards
What is vasculitis?
inflammation of blood vessels, often with ischaemia, necrosis and organ inflam
What is primary vasculitis?
results from an inflammatory response that targets the vessel walla dn has no known cause- sometimes autoimmune
What is secondary vasculitis?
may be triggered by an infection, a drug or a toxin or may occur as part of another inflam disorder or cancer
How is small vessel vasculitis divided?
into conditions asssociated with ANCA and those not
What are the common systemic symptoms in all vasculitides?
fever; malaise, wt loss, fatigue
What are two main causes of large vessel vasculitis?
Takavasu arteritis and Giant Cell arteritis
Who gets Takavaus arteritis?
the under 40s; more common in females and in asian (esp. Japanese) populations
what age gets giant cell arteritis?
over 50s
What are both large vessel vasculitides characterised by pathologically?
granulomatous infiltration of the walls and large vessels
What are the presenting features of the large vessel vasculitides?
bruit- most commonly the carotid; BP difference of extremities; claudication; carotodynia or vessel tenderness; HT
What is temporal arteritis associated with?
PMR
What are the classic symptoms of GCA?
unilateral temporal headache; scalp tenderness and jaw claudication; prominent temporal arteries with reduced pulsation; vision impairmnet
What do you have to be careful with taking biopies in the vasculitides?
there are skip lesions
Why might an MR angiogram or PET CT be done in the large vessel vasculitides?
vessel wall thickening/stenodid/aneurysm or increased metabolic activity
What is the management of large vessel vasculitides?
40-60mg prednisolone (60mg if visual disturbance); grad reduced over 18-24 months.
What are the antibodies associated with the large vessel vasculitides?
there are none!
What is Kawasaki disease?
medium vessel vasculitis which can occur in the coronary arteries where aneurysms can develop
Who is Kawasakis seen in?
children under 5 years
What is polyarteritis nodosa?
necrotising inflam lesions that affect medium arteries at vesssel bifurcations resulting in microanurysm fommration and aneurysms
What organs does Polyarteritis nodosa commonly affect?
skin; gut and kidneys
what disease is PAN often associated with ?
hepB
What was granulomatosis with polyangiitis called previusly?
Wegeners granulomatosis
What was eosinophilic granulomatosis with polyangiitis previosuly called?
Churg Strauss
What is GPA?
granulomatous inflam of the resp tract, small and med vessels. necrotising glowerulonephritis common
What is churg strauss?
eosinophilic granulomatous inflam of the resp tract, small and med vessels and associated with astham
What is microscopic polyangiitis?
necrotising vasculitis with few immune deposits. necrotising glomerulnephritis very common
Who is GPA seen in?
individuals of northern European descent; male-to-female of 1.5:1; 35-55yo
What are the 4 features of GPA?
nasal or oral inflam; abnormal chest radiograph; urinary sediment; granulomatous inflam on biopsy
What are the ENT features of GPA?
sinusitis; nasal crusting; epistaxis; mouth ulcers; deafness; “saddle nose” due to cartilage ischaemia
What are the resp features of GPA?
pulmonary infiltrates; cough; haemoptysis; diffuse alveolar haemorrhage; cavitating nodules on CXR
What are the cutaneous features of GPA?
palpable purpura (non-blanching)- worse in gravity areas- feet, lower limbs; cutaneous ulcers
What are the renal feautres of GPA?
necrotiing glomerulonephritis
What does necrotising glomerulonephritis manifest as?
blood and protein in the urine
How is the nervous system affected in GPA?
mononeuritis multiplex; sensorimotor polyneuropathy; cranial nerve palsy
What happens in mononeuritis multiplex?
peripheral nerves are picked off by inflam in blood vessels eg foot and wrist drop
What are the ocular features of GPA?
conjuctivities; episcleritis; uveitis; optic nerve vasculitis; retinal artery occlusion; proptosis
What is the main difference between EGPA nad GPA?
the presence of late onset asthma and a high eosinophil count
What is seen on hisotlogy of EGPA?
proof of vasculitis; granulomas with extravascular eosinophils
What else is different in EGPA compared to GPA?
ENT involvement is much rarer in EGPA
What are the ANCA antibodies?
a group of autoantibodies against antigens in the cytoplasm of neutrophil granulocytes
What ANCA is associated iwth GPA?
cANCA
What ANCA are MPO and EGPA associated with?
pANCA
What other antibody is associated with GPA?
anti-PR3
What antibody is associated with EGPA and MPA
anti-MPO
why are the antibodies for GPA better than those for EGPA and MPA?
they are more specific
Why are ANCA, anti-PR3 and anti-MPO antibodies useful in monitoring disease?
levels vary with disease actviity
What is the difference between generalised and systemmic AAV?
generlaised is when organs are threatened whereas in systemic vital organs are failing
How is localised/early systemic AAV treated?
methotrexate and steroids
How is generalised/ systemic AAV treated?
cyclophosphamide and steroid
rituximab and steroids then followed by azathioprine for maintenance
How is refractory AAV treated?
IV immunoglobulins
rituximab
What is Henoch-Schonlein Purpura?
an acute immunoglobulin IgA mediated disrorder of the small vessels
Who gets Henoch-Schonlein Purpura?
children aged 2-11
What usually precedes HSP?
an infeection- URTI; pharngeal or GI infections
What is the most common pathogen preceding HSP?
group A strep
When would the preceding illness before HSP be?
1-3 weeks
How does HSP present?
pupuric rash over the buttocks and lower limbs; colicky abdo pain; bloody diarrhoea; joint pain (and swelling) renal involvement
What is the prognosis for HSP?
ususally self-limiting, tend to resolve within 8 weeks, although relapses may occur
