Systemic Vasculitis

Question 1

What is it?

Answer 1

Inflammation of blood vessels
Rare but should be considered if patient presents with multiple symptoms

Can be primary or secondary

Question 2

What symptoms are indicative of SV?

Answer 2

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

Question 3

What is the classification of vasculitis?

Answer 3

Large arteries

Medium arteries

Medium arteries/small vessels (ANCA positive)

Small Vessels

Question 4

What investigations are needed?

Answer 4

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

Question 5

What is the management?

Answer 5

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

Question 6

What are examples of large vessel vasculitis?

Answer 6

GCA

Takayasu’s arteritis

• intermittent limb claudication
• Different BPs in arms
• Carotid bruit
• Aortic regurgitation in 20%

Question 7

What are examples of medium vessel vasculitis?

Answer 7

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

Question 8

What are examples of medium artery/small vessel ANCA positive vasculitis?

Answer 8

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

Question 9

What are examples of small vessel vasculitis?

Answer 9

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