Vasculitis Flashcards
What is the presentation of giant cell arteritis?
- Temporal headache,
- Jaw claudication (pain when chewing)
- Amaurosis fugax (monocular blindness)
- Scalp tenderness,
- Thickened pulsating artery
- May present with polymyalgia rheumatica
What is the age of onset for giant cell arteritis?
60+ years old
What are the main complications of giant cell arteritis?
Permanent monocular blindness - AION
Stroke,
Large vessel vasculitis which can cause stenosis and aneurysms.
What are the investigations for giant cell arteritis?
First line test - Inflammatory markers in particular ESR and FBC, LFTs. (elevated ESR, CRP and platelets and ALP)
Definitive investigations - temporal artery biopsy (every 3-5cm) It will show multinucleated giant cells.
If large vessel involvement is suspected with can be diagnosed by angiography
What is the treatment for giant cell arteritis?
Urgent high dose steroids as soon as diagnosis is suspected.
If no vision less then high dose pred 60mg.
If vision loss then IV methylprednisolone.
May also need to give PPI and bisphosphonates.
Low dose aspirin is also given to reduce risk of stoke.
What treatment can be given to GCA patients who relapse whilst on steroids?
Mycophenolate or methotrexate
What are some examples of ANCA associated small vessel vasculitis
Microscopic Polyangiitis,
Granulomatosis with polyangiitis (Wegner’s)
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)
What is the triad of symptoms for granulomatosis with polyangiitis vasculitis (wegners)?
- Upper respiratory tract involvement. Eg, Chronic sinusitis, epitaxies or saddle nose deformities.
- Lower respiratory tract involvement. Eg, cough, haemoptysis or pleuritis.
- Pauci-immune glomerulonephritis
What are the symptoms of microscopic polyangitis?
- Constitutional symptoms (weight loss, fever, malaise)
- Renal inflammation (necrotizing glomerulonephritis)
- Skin lesions (palpable purpura of lower limbs)
- Peripheral nerve damage
- Lung involvement (haemoptysis)
What is the presentation of eosinophilic granulomatosis with polyangiitis?
Consider with a patient with asthma, nasal polyps and constitutional symptoms.
6 criteria are: asthma, eosinophilia, paranasal sinusitis, pulmonary infiltrates, histological confirmation and neuropathy.
What are the auto-antibodies detected in ANCA vasculitis and what are the methods of detection?
cANCA - targeted against PR3 (Granulomatosis with polyangiitis)
pANCA - targeted against MPO (Eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis)
However not all ANCA vasculitis has positive ANCA (anti-neutrophil cytoplasmic antibodies).
It can be detected via indirect immunofluorescence or ELISA
What are some large vessel vasculitis?
Giant cell,
Takayasu - More likely to affect asian women under 40. Affects branches of aortic arch which can cause weaken/absent UL pulses, carotid bruits and neurological symptoms. Investigated by MRA/CTA and treated with steroids
What are some medium vessel vasculitis?
Kawasaki disease - affects coronary arteries. K for Kids
Polyarteritis nodosa - Causes fibrinoid necrosis and aneurysms. Segmental character causes beads on a string appearance. Causes organ ischaemia. Associated with Hep B
What are some small vessel vasculitides?
ANCA- associated vasculitis.
Immune complex small vessel vasculitis eg, Henoch-scholein purpura or Goodpastures syndrome.
What are the symptoms of kawasaki disease?
Seen mainly in children. It presents with:
- Main complications are coronary artery aneurysm
CRASH and burn
C - conjuntical injection/conjunctivitis
R - Rash
A - adenopathy (cervical)
S - strawberry tongue,
H - Hands/feet erythema
Burn - Fever > 5 days
