Vasculitis Flashcards
Define vasculitis
Inflammation of blood vessels; resulting in vessel wall thickening, stenosis, occlusion and subsequent ischaemia
What is large cell vasculitis?
Primary vasculitis causing chronic granulomatous inflammation of the aorta and its major branches
What are the two main categories of large cell vasculitis?
Temporal (giant cell) arteritis and Takayasu arteritis (TA)
When does large vessel vasculitis onset?
Giant cell- rarely before 50
TA- rarely after 50
Who is affected by TA?
Young women in second and third decades of life- 100x more common in east asia
What does GCA affect?
Temporal arteries but may also (or only) affect the large vessels.
What are the early features of large vessel vasculitis?
Low-grade fever Malaise Night sweats Weight loss Arthlagia Fatigue
What are the later features of large vessel vasculitis?
Claudicant symptoms in upper and lower limbs
If untreated vascular stenosis and aneurysms occur
Result in reduced pulses and bruits
What is elevated in large vessel vasculitis?
ESR, PV, CRP
What will MR angiography detect in large vessel vasculitis?
Thickened vessel walls and stenosis
What is the mainstay of treatment in large vessel vasculitis?
Corticosteroids, start at 40-60mg predisolone and reduce
Steroid sparing agents; methotrexate and azathioprine may be added
What are small to medium vessel vasculitis’ grouped into?
ANCA +ve and ANCA -ve
What are the ANCA associated vasculitis’
Wegeners granulomatosis
Microscopic polyangitis
Churg-strauss
What are the non-ANCA associated vasculitis’
Henoch-schnlein purpura
Cryoglobulinemia
IBD vasculitis
What are the presenting features of small to medium vessel vasculitis?
Fever and weight loss
A raised, non blanching purpuric rash
Arthralgia/arthritis
Mononeuritis multiplex
Glomerulonephritis
Lung opacities on x-ray
Describe Wegeners/granulomaosis with polyangitis
ENT symptoms common, nose bleeds, deafness recurrent sinusitis, nasal crusting (collapse of nose)
Haemoptysis and cavitating lesions on x-ray
What is GPA associated with?
cANCA and PR3 (proteinase-3)
What characterises eosinophilic granulomatosis with polyangitis?
Late onset asthma, rhinitis, raised peripheral blood eosinophil count
Mono neuritis multiplex
What is the most common complication of microscopic polyangitis?
Glomerulonephritis (occurs in 90%)
What investigations are done in microscopic polyangitis?
ESR, PV and CRP (raised)
Anaemia of chronic disease
U+E looking for renal involvement
Anti-neutrophil cytoplasmic antibody (ANCA)
Urinalysis (looking for renal vasculitis)
CXR
Biopsy of an affected area e.g. skin or kidney is often helpful in confirming the diagnosis
How is ANCA associated vasculitis managed?
IV steroids and cyclophosphamide
Describe Henoch-schonlein pupura
Acute immunoglobulin A mediated disorder
Generalised vasculitis involving small vessels of skin, GI, kidneys, joints, lungs, CNS
What are the symptoms of Henoch-schonlein pupura?
Pupuric rash over buttocks and lower limbs, abdominal pain, vomitting, joint pain
What is the treatment for henoch-schonlein pupura?
Self-limiting condition
No specific treatment
Settles weeks/months
