Vasculitis

Question 1

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

What is it most associated with?

What is a characteristic finding?

Answer 1

Eosinophilic granulomatosis with polyangiitis used to be called Churg-Strauss syndrome and is still often referred to by this name. It is a small and medium vessel vasculitis.

It is most associated with lung and skin problems, but can affect other organs such as kidneys.

It often presents with severe asthma in late teenage years or adulthood.

A characteristic finding is elevated eosinophil levels on the full blood count.

Question 2

How do you investigate vasculitis?

p vs. c

Answer 2

Inflammatory markers (CRP and ESR) are usually raised in vasculitis.

Anti neutrophil cytoplasmic antibodies (ANCA) is the blood test to remember for vasculitis:

• p-ANCA (PR3 antibodies) is associated with: Microscopic polyangiitis and Churg-Strauss syndrome [eosinophilic granulomatosis with polyangiitis]
• c-ANCA (MPO antibodies): Wegener’s granulomatosis

Question 3

General Vasculitis Clinical Features

Answer 3

• Purpura. These are purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin.
• Joint and muscle pain
• Peripheral neuropathy
• Renal impairment
• Gastrointestinal disturbance (diarrhoea, abdominal pain and bleeding)
• Anterior uveitis and scleritis
• Hypertension

They are also associated with systemic manifestations of:
Fatigue
Fever
Weight loss
Anorexia (loss of appetite)
Anaemia

Question 4

Vasculitis Management

Answer 4

The management of vasculitis depends on the type. Suspected cases should be referred to a rheumatologist, to guide diagnosis and management. Treatment usually involves a combination of steroids and immunosuppressants.

Steroids can be administered to target the affected area:

• Oral (i.e. prednisolone)
• Intravenous (i.e. hydrocortisone)
• Nasal sprays for nasal symptoms
• Inhaled for lung involves (e.g. Churg-Strauss syndrome)

Immunosuppressants that are used include:

• Cyclophosphamide
• Methotrexate
• Azathioprine
• Rituximab and other monoclonal antibodies

The management of HSP and Kawasaki disease (the types mainly affecting children) is different.

Question 5

Microscopic polyangiitis

What vessels does it affect?

What is the main feature?

Answer 5

Microscopic polyangiitis is a small vessel vasculitis.

The main feature of microscopic polyangiitis is renal failure. It can also affect the lungs causing shortness of breath and haemoptysis.

Question 6

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

What vessels does it affect?

Clinical features

Think about where it affects

Answer 6

Wegener’s granulomatosis is a small vessel vasculitis. It affects the respiratory tract and kidneys.

In the upper respiratory tract it commonly affects the nose causing:
- nose bleeds (epistaxis)
- crusty nasal secretions
- hearing loss
- sinusitis.
A classic sign in exams is the sSADDLE SHAPED NOSE due to a perforated nasal septum. This causes a dip halfway down the nose.

In the lungs it causes a 
- cough
- wheeze 
- haemoptysis. 
A chest xray may show consolidation and it may be misdiagnosed as pneumonia.

In the kidneys it can cause a rapidly progressing glomerulonephritis.

Question 7

Polyarteritis Nodosa

What disease is it associated with?

Clinical features

Diagnosis

Answer 7

It is MOST ASSOCIATED WITH HEPATITIS B

Clinical features

• Cutaneous nodules (hence the name) and ulcers, and testicular pain
• livedo reticularis
• mononeuritis multiplex, sensorimotor polyneuropathy

Diagnosis
- Skin biopsy

Question 8

Kawasaki Disease

What vessels does it affect?

Who does the disease affect?

Clinical features

Key complication

Treatment

Answer 8

Kawasaki disease is a medium vessel vasculitis. It affects young children, typically under 5 years of age. There is no clear cause.

Clinical features are:
- Persistent high fever > 5 days
- Erythematous rash
Bilateral conjunctivitis
- Erythema and desquamation (skin peeling) of palms and soles
- “Strawberry tongue” (red tongue with prominent papillae)

A key complication is coronary artery aneurysms.

Treatment is with aspirin and IV immunoglobulins.

Question 9

Takayasu’s arteritis

What vessels does it affect? In particular which vessel does it tend to affect?

What does it cause the vessels it affects to do?

Clinical features
At what age does it tend to present

How is it diagnosed?

Answer 9

Takayasu’s arteritis is a form of large vessel vasculitis. It mainly affects the aorta and it’s branches. It also affect the pulmonary arteries.

These large vessels and their branches can swell and form aneurysms or become narrowed and blocked. This leads to it’s other name of “pulseless disease”.

It usually presents:
- before the age of 40 years with non-specific systemic symptoms, such as fever, malaise and muscle aches, or with more specific symptoms of arm claudication or syncope.

It is diagnosed using CT or MRI angiography. Doppler ultrasound of the carotids can be useful in detecting carotid disease.

Question 10

Giant Cell arteritis

Commonest type

Biopsy

Management

Answer 10

Temporal arteritis is commonest type

Granulomatous lesions may be seen on biopsy

HIGH dose (60mg prednisolone) to prevent blindness
(If vision loss has happened: IV methylprednisolone)

+ PPI for gastroprotection

+ Aspirin can be used to reduce the risk of thromboembolism in vasculitis