Vasculitis Flashcards
what is vasculitis
inflammation of blood vessels, often with ischemia, necrosis and organ inflammation- presentation depends on the size and location affected
describe primary vasculitis
results from inflammatory response that targets the vessels walls and has unknown cause- sometimes autoimmune
describe secondary vasculitis
may be triggered by an infection, a drug or toxin or may occur as part of another inflammatory disorder or cancer
what happens to the vessel in vasculitis
activated dendritic cell release activation of T cells and vascular inflammation. activated T cells promotes inflammation, granuloma formation and macrophage activation. macrophages produce a variety of mediators that lead t progressive vascular inflammation, endothelial damage, narrowing of lumen
what are the different classifications of vasculitis
split on the size of the vessels
small vessel vascultits split into anca and non anca associated and
what systemic symptoms are common to all vasculitides
fever, malaise, weight loss, fatigue
what are the two main causes of large vessel vasculitis and who do they usually affect
takayasu arteritis (TA)- females under 40 giant cell arteritis (GCA)- over 40
what type of reactions is seen in large vessel vasculitis
granulomatous infiltrations of the walls of the large vessel
what are the presenting features of large vessel vasculitis
bruit (most commonly carotid artery)
blood pressure difference of extremenities
cluadication
what is temporal arteritis associated with
polymyalgia rhematica
what are the symptoms of temporal arteritis
unilateral temporal headache, scalp tenderness and jaw claudication
temporal arteries prominent with reduced pulse
what is there a risk of in temporal arteritis
blindness- ischaemia of the optic nerve
what investigations can be done into temporal arteritis
ESR, PV, CRP will be raised
temporal artery biopsy (skip lesion might cause false negative)
MR angiogram or PET CT
What is the management for temporal arteritis
40-60mg prednisolone/steroid sparing agent
increased dose if visual loss
what is AAV
anca associated vasculitis
AAV with granulomas present=?
ask about asthma and eosinophilia present
if yes= churg strauss syndrome
if no= wengers granolmatosis
AAV without granulomas present=?
microscopic molyangitis
what is eosinophillic granulomatous with polyagniitis (EGPA) (churg-strauss syndrome)
eosinophilic granulomatous inflammation of resp tract, small and medium vessels
what is microscopic polyangiitis (MPA)
necrotising vasculitis with few immune deposits
what is granulomatosis with polyangitis (GPA) (wegener’s granulomatous)
granulomatous inflammation of respiratory tract, small vessels
what is the diagnostic signs of granulomatosis with polyangitis
nasal or oral inflammation
abnormal chest x ray
urinary sediment
granulomatous inflammation on biopsy
what are the ENT features of granulomatosis with polyangitis
sinusitis nasal crusting epistaxis mouth ulcers sensorineural deafness otitis media and deafness saddle nose due to cartilage ischaemia
what are the respiratory symptoms of granulomatosis with polyangitis
pulmonary infiltrates cough haemoptysis diffuse alveolar haemorrhage cavitating nodules on CXR
what are the cutaneous reactions of granulomatosis with polyangitis
palpable purpura
cutaneous ulcers
what are the renal signs of granulomatosis with polyangitis
necrotising glomerulonephritis
what are the nervous signs of granulomatosis with polyangitis
Mononeuritis multiplex
Sensorimotor polyneuropathy
Cranial nerve palsies
what are the occular signs of granulomatosis with polyangitis
Conjunctivitis Episcleritis Uveitis Optic nerve vasculitis Retinal artery occlusion Proptosis
what is the main difference of eosinophillic granulomatosis with polyangitis (EGPA) and granulomatosis with polyangitis (PGA)
EPA has late onset asthma and a high eosinophil count
what are the diagnostic features of EGPA
asthma, eosinophilia of more than 10% in blood, paranasal sinusitis, pulmonary infiltrates, histological proof of vasculitis with extravascular eosinophils, mononeuritis multiplex or polyneuropathy
what does ANCA stand for
anti-neutrophil cytoplasmic antibodies
what are ANCAs
auto-antibodies against antigens in the cytoplasm of neutrophils granulocytes
what is used to differentiate between anca and non anca
immunofluoresence
what types of anca in GPA and MPO (microscopic polyangitis) and EGPA
cANCA- GPA
pANCA- MPO and EGPA
what immune process is associated with AAV
the formation of immune complexes
what autoantibodies are associated with GPA
cANCA and anti-PR3
what is the treatment for AAV
localised/ early systemic- methotrexate + steroids
generalised/ systemic- cyclophosphamide (or rituximab) + steroids
refractory- IV immunoglobulins, rituximab
is recurrent sinusitis a common feature of EGPA
yes
in non ANCA small vessel vasculitis how are the sub types classified
IgA dominant immune deposits?
if yes- henoch-schlein purpura
if no- check for serum cryoglobulin
what are the types of non IgA dominant immune deposit non ANCA small vessels vasculitis
if serum cryoglobulin- cryoglobulinemia
if non other non ANCA vasculitis e.g. IBD vasculitis
describe henoch-schonlein purpura (HSP)
an acute IgA mediated disorder causing generalised vasculitis involving the small vessels of the skin, GI tract, kidneys, joints and rarely lungs and CNS
who gets HSP
children aged 2-11
what is HSP commonly predisposed by
upper resp tract infection (usually group A strep)
what is the presentation of HSP
purpuric rash typically over buttocks and lower limbs
colicky abdo pain
bloody diarrhoea
joint pain +/- swelling
renal involvement
how is HSP managed
usually self limiting
tend to resolve in 8 weeks
relapses may occur
screen for renal involvement
