Systemic Vasculitis Flashcards
What is it?
Inflammation of blood vessels
Rare but should be considered if patient presents with multiple symptoms
Can be primary or secondary
What symptoms are indicative of SV?
General
- weight loss
- fever
- malaise
- myalgia
- arthralgia
Skin
- palpable purpura
- ulceration
GI
- mouth ulcers
- abdo pain
- diarrhoea
Resp
- haemoptysis
- dyspnoea
ENT
- epistaxis
- crusting
Cardiac- chest pain
Neuropathies
What is the classification of vasculitis?
Large arteries
Medium arteries
Medium arteries/small vessels (ANCA positive)
Small Vessels
What investigations are needed?
Bedside
- Urine dip- renal involvement as determines prognosis
- BP=essential
Bloods
- FBC- leukocytosis in primary disease/infection
- LFTs- hep is secondary cause
- Inflammatory markers
- C-ANCA associated with wegeners
- p-ANCA assocaited with churg strauss and microscopic polyangiitis
Tissue biopsy to confirm diagnosis
What is the management?
Depends on size of vessel involved
Corticosteroids=mainstay
-reduce dose over 12 months
-IVIG for kawasaki
-Cyclophosphamide often used in ANCA positive disease to induce remission- can cause infertility
Plasma exchange in life threatening conditions
What are examples of large vessel vasculitis?
GCA
Takayasu’s arteritis
- intermittent limb claudication
- Different BPs in arms
- Carotid bruit
- Aortic regurgitation in 20%
What are examples of medium vessel vasculitis?
Kawasaki
-fever, rash, lymphadenopathy and palmar erythema in children
Polyarteritis Nodosa
- associated with HepB infection
- HTN
- livedo reticularis
- testicular pain (more common in men)
- haematuria and renal failure
- sensorimotor polyneuropathy
What are examples of medium artery/small vessel ANCA positive vasculitis?
Wegeners Granulomatosis
- epistaxis
- nasal crusting
- rapidly progressing glomerulonephritis
- vasculitic rash
- saddle shaped nose
Churg Strauss Syndrome
- associated with late onset asthma and atopy
- paranasal sinusitis
- PANCA +ve in 60%
Microscopic polyangitis
- renal failure= haematuria, proteinuria and raised Creatinine
- dyspnoea
- haemoptysis
- palpable purpura
What are examples of small vessel vasculitis?
HSP
- palpable purpuric rash over buttocks and extensor surfaces of legs and arms
- abdo pain
- polyarthritis
- renal failure
Cryoglobulinaemia
- associated with HepC
- rash
- neuropathy
- arthralgia