Vasculitis Flashcards
What are the different categories of vasculitis?
Large vessel Medium vessel Small vessel: - ANCA associated - Immune complex
What are the large vessel vasculitides?
Takayasu arteritis
Giant cell/temporal arteritis
What are the medium vessel vasculitides?
Polyarteritis nodosa
Kawasaki disease
What are the ANCA-associated small vessel vasculitides?
Microscopic polyangitis
Granulomatosis with polyangitis
Eosinophilic granulomatosis with polyangitis
What are the immune complex small vessel vasculitides?
Cryoglobulinaemic vasculitis
IgA vasculitis (Henoch-Schonlein)
Hypocomplementemic urticarial vasculitis
What is the pathology of large vessel vasculitis?
Chronic granulomatous inflammation of aorta + major branches
Who gets Takayasu arteritis?
Women, < 40
More common in Asian population
Who gets GCA?
> 50 years old
What are the clinical features of GCA?
Headache - continuous, temporal/occipital
Scalp tenderness e.g. hair combing
Jaw claudication - fatigue/discomfort on chewing/speaking (pathognomonic of GCA)
Visual loss
What causes jaw claudication in GCA?
Ischaemia of maxillary artery
What causes the visual loss in GCA?
Amaurosis fugax - loss of blood supply to eye
-> swollen optic disc
Permanent in about 20%
What might you see on examination of someone with GCA?
Tender, enlarged, non-pulsatile temporal arteries
What is the gold standard for diagnosis of GCA and what would it show?
Temporal artery biopsy –> asap
- 100% specificity but only 15-40% sensitivity due to patchy involvement of artery
Mononuclear infiltration or granulomatous inflammation, usually with multinucleate giant cells
Which investigation might be helpful if biopsy negative but clinical suspicion of GCA?
Temporal artery USS
What is the treatment for GCA?
Rapid corticosteroids to avoid loss of vision –> do not delay to wait for biopsy
- prednisolone 40mg if no vision impairment
- 60mg if vision affected
Reduce dose gradually over about 2 years
Which constitutional symptoms are often seen with large vessel vasculitides?
Fever Malaise Night sweats Weight loss Arthralgia Fatigue
What are the clinical features of Takayasu arteritis?
Claudication symptoms in both upper and lower limbs
Reduced pulses
Difference in BP between limbs
Artery bruits
Which investigations should be done to diagnose TA?
Raised inflammatory markers
BP
MR angiogram –> thick vessel walls + stenosis
How is TA treated?
40-60mg prednisolone
+/- methotrexate or azathioprine
What are the common features of small vessel vasculitides?
Fever + weight loss Raised, non-blanching purpuric rash Arthralgia/arthritis Mononeuritis multiplex Glomerulonephritis Lung opacities on CXR
What is the old name for GPA?
Wegener’s granulomatosis
What are the clinical features of GPA?
Common features of small vessel vasculitis + ENT symptoms: - nose bleeds - deafness - recurrent sinusitis - nasal crusting - 'saddle nose' (collapsing of cartilage) Haemoptysis + caveatting lesions on CXR
Which blood markers are associated with GPA?
cANCA + PR3
What is the old name for EGPA?
Churg-Strauss syndrome
What are the clinical features of EGPA?
Late onset asthma Rhinitis High peripheral blood eosinophil count Mononeuritis multiplex common Other features similar to GPA
Which blood markers are associated with EGPA?
pANCA
What are the clinical features of microscopic polyangiitis (MPA)?
Similar to other small vessel vasculitis
Glomerulonephritis very common (in up to 90%)
Which blood markers are associated with MPA?
pANCA + MPO
What is the management of ANCA associated vasculitis?
Localised/early systemic: --> methotrexate + steroids Generalised/systemic (most patients): --> IV steroids + cyclophosphamide Refractory: --> IV Ig + rituximab
What is Henoch-Schonlein Purpura?
Acute IgA mediated vasculitis primarily affecting GI tract, kidneys + joints
Who gets HSP?
Children age 2-11
What is the classic presentation of HSP?
URTI (group A strep), 1-3 weeks later –>
- purpuric rash on buttocks/legs
- colicky abdominal pain
- bloody diarrhoea
- joint pain/swelling
- glomerulonephritis in 50%
Which investigations should be done in HSP?
ESSENTIAL to do urinalysis to check for renal involvement
What is the management + prognosis of HSP?
Symptomatic - usually self-limiting, resolves within 8 weeks
