Vasculitis Flashcards
what is vasculitis
= inflammation of the blood vessels, can be arteries, veins, capillaries or venules
what can vasculitis lead to
inflammation, ischaemia and/or necrosis of the tissue being supplied by the vessel
describe what primary vasculitis results from
results from inflammatory response that targets the vessel walls with no known cause
describe what secondary vasculitis may be triggered by
infection, drugs, toxins, or may occur as part of other inflammatory disorder or cancer
what cells are thought to be involved in the initial pathophysiology of vasculitis
dendritic cells(antigen presenting cells)
what are some of the common systemic symptoms seen in vasculitis
fever, malaise, weight loss and fatigue
what are the 3 main broad categories vasculitis is classified into
large cell vasculitis, small cell vasculitis and ANCA-associated vasculitis
what are the main types of large cell vasculitis
takayasu arteries(TA) and giant cell/temporal arteritis(GCA)
what pathology is seen in large cell vasculitis
granulomatous infiltration of the walls of the large vessels
what group of people is takayasu arteritis more common in
females, under 40, more common in asian population
what are some of the clinical features of takayasu arteritis
claudication(esp. of arm so have sore arm muscles), bruit(most commonly carotids), blood pressure differences on each side, can be pulseless
what is the main investigation used for takayasu arteritis
angiogram
what group of people does giant cell/temporal arteritis most commonly affect
over 50, most commonly over 60
describe the clinical features of giant cell/temporal arteritis
unilateral acute temporal headache, scalp tenderness, temporary visual disturbance/blindness, jaw claudication
what affect can giant cell/temporal arteritis have on the temporal arteries
may be prominent with reduced pulse
what condition does giant cell/temporal arteritis strongly associated with
polymyalgia rheumatica
what blood tests are done for giant cell/temporal arteritis
ESR or plasma viscosity raised, CRP raised
95% have either 1 or both markers raised
what is the gold standard investigation for giant cell/temporal arteritis if inflammatory markers not helpful
temporal artery biopsy
however can be skip lesions so may be false negative
what imaging can be done for giant cell/temporal arteritis
ultrasound scan, PET CT or CT angiogram
what 2 categories is small vasculitis broadly categorised into
ANCA-associated small vessel vasculitis and Non-ANCA-associated small vessel vasculitis
what different aetiological factors are involved in ANCA-associated small vessel vasculitis
genetic factors(eg HLA genes), environmental(eg silica), drugs(eg hydralazine), bacteria, cells, molecules(eg ANCA)
what 3 different conditions is ANCA-associated small vessel vasculitis further classified into, due to different pathology
granulomatous with polyangiitis(GPA), eosinophilic granulomatosis with polyangiitis(EGPA), microscopic polyangiitis(MPA)
describe the pathology of GPA ANCA-associated small vessel vasculitis
granulomatous inflammation of respiratory tract, small and medium vessels
necrotising glomerulonephritis common
describe the pathology of EGPA in ANCA-associated small vessel vasculitis
eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels
associated with asthma
describe the pathology of MPA ANCA-associated small vessel vasculitis
necrotising vasculitis with few immune deposits
necrotising glomerulonephritis very common
what group of people is granulomatous with polyangiitis(GPA) ANCA-associated small vessels vasculitis most commonly seen in
more common northern Europe, 35-55 years old, male : female is 1.5:1
what are some of the ENT features seen in GPA
sinusitis, nasal crusting, mouth ulcers, epistaxis, otitis media and deafness
what respiratory symptoms can be seen in GPA
cough, haemoptysis, pulmonary infiltrates, diffuse alveolar haemorrhage, cavitating nodules on CXR
what are some ocular symptoms that may be seen in GPA(rare)
conjunctivitis, uveitis, episcleritis
what cutaneous features can be seen in GPA
palpable purpura, cutaneous ulcers
what renal feature is see in GPA
necrotising glomerulonephritis
causes lethargy, fatigue
what affect can GPA have on the nervous system
most commonly sensorimotor polyneuropathy
how do the clinical features of EGPA compare to GPA
many similar clinical features
main difference = late onset asthma, high eosinophil count and ANCA specificity
what clinical features are commonly caused by microscopic polyangiitis
renal impairment
what is the most common non-ANCA associated small vessel vasculitis
Henoch-Schonlein Purpura(HSP)
what mediates HSP
IgA
describe what Henoch-Schonlein Purpura (HSP) vasculitis is
generalised vasculitis involving the small vessels of the skin, GI tract, kidneys, joints,
what group of people is HSP most common in
children 2-11 years old
what infections commonly(75%) precede HSP, and what organism is most commonly the cause
UTI, GI infection, pharyngeal infection
organism = group A streptococcus
(usually 1-3 weeks before HSP)
what is the clinical presentation of HSP
purpuric rash typically on lower limbs and buttocks, colicky abdominal pain, bloody diarrhoea, joint pain/swelling, renal involvement(50%)
