Vasculitis Flashcards
GCAr Polyarteritis nodosa Microscopic polyangiitis
What are the large vessel vasculitides?
GCA
Takayasu’s arteritis
Buergers disase
What are the medium vessel vasculitides?
Polyarteritis nodosa
Kawasaki disease
What are the small vessel vasculitides
- ANCA associated:
- microscopic polyangiitis
- Wegeners granulamtoiss
- Churg strauss - Immune complex vasculitis:
- goodpastures
- IgA vasculitis (henoch-schonlein)
- Cryoglobulinaemic vasculitis
How do a lot of vasculitides present?
Fatigue + raised ESR/CRP
What is it appropriate to consider if a presentation doesn’t fit into any category?
Ca associated
What is the general treatment of large vessel vasculitides?
steroids
can add steroid sparing agents
What is the general treatment of medium/small vessel vasculitides?
immunosuppression
steroids +/- sparing agent
What is GCA also known as?
temporal arteritis
What is GCA commonly associated w?
PMR
What age is GCA more common in?
> 50
What are the sx of GCA?
- HEADACHE - new onset usually unilateral in temporal region but can be bilateral and diffuse
- TEMPORAL ARTERY - tender, thickened or nodular, may be red w absence or reduced pulse
- SCALP TENDERNESS
- Tongue/jaw claudicaton
- Amaurosis fugax
What is an irreversible complication of GCA?
Unilateral blindness (ophthalmic artery)
What are the extra cranial sx of GCA?
malaise WL Morning stiffness SOB Unequal/weak pulses
How would you make a diagnosis of GCA?
≥3 of:
- > 50yrs
- New headache
- Temporal artery tenderness or reduced pulse
- ESR >50
- Abnormal artery biopsy
What is seen on biopsy in GCA?
Necrotising arteries w mononuclear infiltrate or granulomatous inflammation
What blood tests changes would support a diagnosis of GCA?
↑platelets
↑CRP
↑ALP
↓Hb
What is the management of GCA
- Prednisolone ASAP 60mg/d PO
or - IV methylprednisolone if evolving visual loss or Hx of amaurosis fugax
Remember to give:
PPI, bisphosphonates, Ca2+ w cholecalciferol + consider aspirin
How long do ppl tend to have GCA before remission?
2yrs
When should prednisolone dose be reduced in GCA?
when sx resolve and ESR ↓
what is polyarteritis nodosa (PN)?
Necrotising vasculitis causing aneurysms + thrombosis in medium sized arteries, leading to infarction in affected organs w severe systemic sx
Which sex is more affected in PN?
m>f 2:1
What may PN be associated w?
Hep B
What are the systemic sx of PN?
fever
malaise
WL
arthralgia
What are the skin features of PN?
Rash
‘punched out’ ulcers
nodules
levidoreticularis
What are the renal features of PN
HTN
RF
Haematuria
proteinuria
What are the neurological sx of pN
mononeuritis multiplex
sensori-motor polyneuropathy
What are the cardiac features of pn
angina/MI
HF
Pericarditis
What are the GI features of PN
pain or perforation
malabsorption
What are the GU features of PN
orchitis
testicular pain
What organs are usually spared in pn
lungs
What tests would u do in PN?
- FBC: ↓Hb, ↑WCC, mild eosinophilia
- ↑ESR/CRP
- ANCA -ve
- Renal biopsy or angiography
What is the rx of polyarteritis nodosa?
i. control BP
ii. Steroids - mild cases
iii. Steroid sparing agents if severe
What is microscopic polyangiitis (MP)?
necrotising vasculitis affecting small and medium vessels
What are the sx of MP?
- rapidly progressive GN
- pulmonary haemorrhage
How is MP diagnosed?
pANCA +ve
What is the treatment of MP
steroids + e.g. MTX
Maintenance - MTX, ritux, azathioprine