Vasculitis

Question 1

what is vasculitis

Answer 1

= inflammation of the blood vessels, can be arteries, veins, capillaries or venules

Question 2

what can vasculitis lead to

Answer 2

inflammation, ischaemia and/or necrosis of the tissue being supplied by the vessel

Question 3

describe what primary vasculitis results from

Answer 3

results from inflammatory response that targets the vessel walls with no known cause

Question 4

describe what secondary vasculitis may be triggered by

Answer 4

infection, drugs, toxins, or may occur as part of other inflammatory disorder or cancer

Question 5

what cells are thought to be involved in the initial pathophysiology of vasculitis

Answer 5

dendritic cells(antigen presenting cells)

Question 6

what are some of the common systemic symptoms seen in vasculitis

Answer 6

fever, malaise, weight loss and fatigue

Question 7

what are the 3 main broad categories vasculitis is classified into

Answer 7

large cell vasculitis, small cell vasculitis and ANCA-associated vasculitis

Question 8

what are the main types of large cell vasculitis

Answer 8

takayasu arteries(TA) and giant cell/temporal arteritis(GCA)

Question 9

what pathology is seen in large cell vasculitis

Answer 9

granulomatous infiltration of the walls of the large vessels

Question 10

what group of people is takayasu arteritis more common in

Answer 10

females, under 40, more common in asian population

Question 11

what are some of the clinical features of takayasu arteritis

Answer 11

claudication(esp. of arm so have sore arm muscles), bruit(most commonly carotids), blood pressure differences on each side, can be pulseless

Question 12

what is the main investigation used for takayasu arteritis

Answer 12

angiogram

Question 13

what group of people does giant cell/temporal arteritis most commonly affect

Answer 13

over 50, most commonly over 60

Question 14

describe the clinical features of giant cell/temporal arteritis

Answer 14

unilateral acute temporal headache, scalp tenderness, temporary visual disturbance/blindness, jaw claudication

Question 15

what affect can giant cell/temporal arteritis have on the temporal arteries

Answer 15

may be prominent with reduced pulse

Question 16

what condition does giant cell/temporal arteritis strongly associated with

Answer 16

polymyalgia rheumatica

Question 17

what blood tests are done for giant cell/temporal arteritis

Answer 17

ESR or plasma viscosity raised, CRP raised

95% have either 1 or both markers raised

Question 18

what is the gold standard investigation for giant cell/temporal arteritis if inflammatory markers not helpful

Answer 18

temporal artery biopsy

however can be skip lesions so may be false negative

Question 19

what imaging can be done for giant cell/temporal arteritis

Answer 19

ultrasound scan, PET CT or CT angiogram

Question 20

what 2 categories is small vasculitis broadly categorised into

Answer 20

ANCA-associated small vessel vasculitis and Non-ANCA-associated small vessel vasculitis

Question 21

what different aetiological factors are involved in ANCA-associated small vessel vasculitis

Answer 21

genetic factors(eg HLA genes), environmental(eg silica), drugs(eg hydralazine), bacteria, cells, molecules(eg ANCA)

Question 22

what 3 different conditions is ANCA-associated small vessel vasculitis further classified into, due to different pathology

Answer 22

granulomatous with polyangiitis(GPA), eosinophilic granulomatosis with polyangiitis(EGPA), microscopic polyangiitis(MPA)

Question 23

describe the pathology of GPA ANCA-associated small vessel vasculitis

Answer 23

granulomatous inflammation of respiratory tract, small and medium vessels
necrotising glomerulonephritis common

Question 24

describe the pathology of EGPA in ANCA-associated small vessel vasculitis

Answer 24

eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels
associated with asthma

Question 25

describe the pathology of MPA ANCA-associated small vessel vasculitis

Answer 25

necrotising vasculitis with few immune deposits | necrotising glomerulonephritis very common

Question 26

what group of people is granulomatous with polyangiitis(GPA) ANCA-associated small vessels vasculitis most commonly seen in

Answer 26

more common northern Europe, 35-55 years old, male : female is 1.5:1

Question 27

what are some of the ENT features seen in GPA

Answer 27

sinusitis, nasal crusting, mouth ulcers, epistaxis, otitis media and deafness

Question 28

what respiratory symptoms can be seen in GPA

Answer 28

cough, haemoptysis, pulmonary infiltrates, diffuse alveolar haemorrhage, cavitating nodules on CXR

Question 29

what are some ocular symptoms that may be seen in GPA(rare)

Answer 29

conjunctivitis, uveitis, episcleritis

Question 30

what cutaneous features can be seen in GPA

Answer 30

palpable purpura, cutaneous ulcers

Question 31

what renal feature is see in GPA

Answer 31

necrotising glomerulonephritis | causes lethargy, fatigue

Question 32

what affect can GPA have on the nervous system

Answer 32

most commonly sensorimotor polyneuropathy

Question 33

how do the clinical features of EGPA compare to GPA

Answer 33

many similar clinical features | main difference = late onset asthma, high eosinophil count and ANCA specificity

Question 34

what clinical features are commonly caused by microscopic polyangiitis

Answer 34

renal impairment

Question 35

what is the most common non-ANCA associated small vessel vasculitis

Answer 35

Henoch-Schonlein Purpura(HSP)

Question 36

what mediates HSP

Answer 36

IgA

Question 37

describe what Henoch-Schonlein Purpura (HSP) vasculitis is

Answer 37

generalised vasculitis involving the small vessels of the skin, GI tract, kidneys, joints,

Question 38

what group of people is HSP most common in

Answer 38

children 2-11 years old

Question 39

what infections commonly(75%) precede HSP, and what organism is most commonly the cause

Answer 39

UTI, GI infection, pharyngeal infection organism = group A streptococcus (usually 1-3 weeks before HSP)

Question 40

what is the clinical presentation of HSP

Answer 40

purpuric rash typically on lower limbs and buttocks, colicky abdominal pain, bloody diarrhoea, joint pain/swelling, renal involvement(50%)