Vasculitis Flashcards
What is vasculitis
inflammation of blood vessels
generally, how is vasculitis classified
histological type of inflammation
size of vessel segment involved
the distribution of involved vessels
2 types of large vessel vasculitis
Giant Cell Arteritis
Takayasu arteritis
2 types of medium vessel vasculitis
Kawasaki disease
Polyarteritis nodosa
Types of small vessel vasculitis
ANCA-associated
Non- ANCA associated
Types of ANCA-associated small vessel vasculitis
Microscopic polyangitis
Granulomatosis with polyangitis (Wegener)
Eosinophilic granulomatosis with polyangitis (Churg-Strauss)
Types of non-ANCA associated small vessel vasculitis
Cryoglobulinemic vasculitis
IgA vasculitis (Henoch-Schonlein)
what vessels do Large vessel arteritis affect
aorta and its major branches
What is the main difference between Giant Cell arteritis and Takaysu Arteritis
Age of onset
GCA occurs rarely before 50 years
TA occurs rarely after 50 years
Describe pathology of GCA
transmural inflammation of the intima, media and adventitia of affected arteries.
Patch infiltration by lymphocytes, macrophages, and multinucleated giant cells.
Vessel wall thickening - arterial luminal narrowing, resulting is ischaemia.
condition commonly associated with GCA
polymyalgia rheumatica
artery most commonly affected by GCA
temporal artery
presentation of GCA
visual disturbances
headache
jaw claudication
scalp tenderness (esp with hair combing)
constitutional symptoms:
fatigue
malaise
fever
what symptom of GCA is the most significant causes of morbidity
visual disturbances
what causes the jaw claudication seen in GCA
ischaemia of the maxillary artery
visual disturbances seen in GCA
unilateral visual blurring
unilateral visial loss
painless
diplopia
definitive test for GCA
temporal artery biopsy
main problem with temporal artery biopsy as diagnostic test for GCA
it has 100% specificity, but only 15-40% sensitivity (patchy involvement of artery, so some segments may be normal)
typical biopsy findings in GCA
mononuclear infiltration or granulomatous inflammation with multinucleated giant cells
Mx of GCA
40mg pred if no visual symptoms
60mg pred if visual symptoms
when should treatment of GCA be started
as soon as there is clinical suspicion - dont wait for biopsy!!
inflammatory markers in GCA
plasma viscosity
ESR
CRP
- all elevated
most common group of people to get Takayasu Arteritis
females <40 years and Asian populations
Consequence of untreated large vessel vasculitis
vascular stenosis and aneurysms
- results in reduced pulses and (carotid) bruits
Tx Takayasu Arteritis
same as for GCA
40-60mg prednisolone daily
What age group gets Kawasaki Disease
almost exclusively infants <5 years
What is Kawasaki Disease
medium vessel vasculitis, that is a self-limiting acute febrile illness lasting around 5 days
presentation of Kawasaki Disease
fever polymorphic rash injected eyes mucosal erythema strawberry tongue
Tx Kawasaki Disease
IVIG +/- aspirin
What vessels does medium vessel vasculitis most commonly affect
skin
gut
kidneys
What is Polyarteritis Nodosa
medium vessel vasculitis, with necrotising inflammatory lesions that affect arteries at vessel bifurcations resulting in microaneurysm formation in affected organs
Shared features of small vessel vasculitis
fever & weight loss raised, non blanching purpuric rash arthralgia/arthritis mononeuritis multiplex glomerulonephritis lung opacities on xray
What is Granulomatosis with Polyangiitis (GPA/Wegeners)
systemic vasculitis affecting small and medium sized vessels.
granulomatous inflammation involves the upper and lower respiratory tract, with necrotising glomerulonephritis
Symptoms of GPA
ENT features -
nose bleeds, deafness, recurrent sinusitis, nasal crusting, “saddle nose” due to cartilage ischaemia
Respiratory symptoms -
cavitating lesions on xray
(also shared symptoms of small vessel vasculitis)
What are ANCA
Anti-neutrophil cytoplasmic antibodies
A group of antibodies against antigens in the cytoplasm of neutrophil granulocytes
ANCA pattern in GPA
cANCA
What is Eosinophilic Granulomatosis with Polyangiitis (EGPA/Churg-Strauss)
systemic vasculitis affecting small and medium sized vessels.
eosinophilic and granulomatous inflammation involving the respiratory tract.
associated with asthma and eosinophilia
ANCA pattern in EGPA
pANCA
which small vessel vasculitis is most associated with PR3-ANCA
GPA
small vessel vasculitis most associated with MPO-ANCA
MPA
What is microscopic polyangiitis (MPA)
systemic vasculitis of small and medium vessels - not associated with granuloma formation.
most important feature of MPA
glomerulonephritis
Ix of small vessel vasculitis
inflammatory markers FBC - anaemia of chronic disease U&E - renal involvement ANCA Urinalysis (renal vasculitis) CXR Biopsy of affected area
Mx of ANCA-associated vasculitis
IV steroids and cyclophosphamide
What is Henoch-Schonlein purpura?
Non-ANCA associated small vessel vasculitis
An acute IgA mediated disorder
Most common organs affected by Henoch-Schonlein purpura?
smalls vessels of skin
GI tract
kidneys
joints
Presentation of Henoch-Schonlein purpura?
URTI precedes symptoms by a few weeks purpuric rash over buttocks and lower limbs abdo pain vomiting joint pain
Tx of Henoch-Schonlein purpura?
self-limiting
symptoms tend to resolve over 8 weeks
most common organism to cause Henoch-Schonlein purpura?
Group A Strep
most common age group for HSP
children
what disease is Polyarteritis Nodosa strongly associated with
Hepatitis B
