Vasculitis

Question 1

What is vasculitis?

Answer 1

Vasculitis is the name for inflammation of the blood vessels. There are many different types of vasculitis that affect different sizes of blood vessel.

They are categorised based on whether they affect small vessels, medium sized vessels or large vessels. They each have some unique features that will help you spot them in exams.

Question 2

Types of Vasculitis Affecting The Small Vessels

Answer 2

Henoch-Schonlein purpura
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Microscopic polyangiitis
Granulomatosis with polyangiitis

Question 3

Types of Vasculitis Affecting The Medium Sized Vessels

Answer 3

Polyarteritis nodosa
Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
Kawasaki Disease

Question 4

Types of Vasculitis Affecting The Large Vessels

Answer 4

Giant cell arteritis
Takayasu’s arteritis

Question 5

There are some generic features that apply to most types of vasculitis. These are things that should make you think about a possible vasculitis:

Answer 5

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

Question 6

Systemic manifestations might include:

Answer 6

Fatigue
Fever
Weight loss
Anorexia (loss of appetite)
Anaemia

Question 7

Tests for vasculitis

Answer 7

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

Anti neutrophil cytoplasmic antibodies (ANCA) is the blood test to remember for vasculitis. If you remember this alone you will be able to answer many questions on vasculitis.

There are two type of ANCA blood tests: p-ANCA and c-ANCA. P-ANCA are also called anti-MPO antibodies. C-ANCA are also called anti-PR3 antibodies. These different ANCA tests are associated with different types of vasculitis:

• p-ANCA (MPO antibodies): Microscopic polyangiitis and Churg-Strauss syndrome
• c-ANCA (PR3 antibodies): Granulomatosis with polyangiitis

Question 8

Management of vasculitis?

Answer 8

The management of vasculitis depends on the type. Suspected cases should be referred to a specialist, usually 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 9

What is Henoch-Schonlein purpura?

Answer 9

Henoch-Schonlein Purpura (HSP) is an IgA vasculitis that commonly presents with a purpuric rash affecting the lower limbs or buttocks in children. Inflammation occurs due to immunoglobulin A deposits in the blood vessels of affected organs such as the skin, kidneys and gastro-intestinal tract. The condition is often triggered by an upper airway infection (e.g. tonsillitis) or a gastroenteritis. It is most common in children under the age of 10 years. The rash is caused by inflammation and leaking of blood from small blood vessels under the skin, forming purpura.

Question 10

What are the classic features of Henoch-Schonlein purpura?

Answer 10

The four classic features are purpura (100%), joint pain (75%), abdominal pain (50%) and renal involvement (50%).

Question 11

What is the renal involvement in Henoch-Schonlein purpura?

Answer 11

HSP affects the kidneys in about 50% of patients, causing an IgA nephritis.

Question 12

Management of Henoch-Scholein purpura?

Answer 12

Management is typically supportive, such as simple analgesia, rest and proper hydration. The benefits of steroids are unclear.

The abdominal pain usually settles within a few days. Patients without kidney involvement can expect to fully recover within 4-6 weeks. A third of patients have a recurrence of the disease within 6 months. 1% of patients will go on to develop end stage renal failure.

Question 13

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)?

Answer 13

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 14

Microscopic polyangiitis

Answer 14

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 15

Granulomatosis with polyangiitis

Answer 15

Granulomatosis with polyangiitis is a small vessel vasculitis. It was previously known as Wegener’s granulomatosis. It affects the respiratory tract and kidneys.

In the upper respiratory tract, it commonly affects the nose causing nose bleeds (epistaxis) and crusty nasal secretions, ears causing hearing loss and sinuses causing sinusitis. A classic sign in exams is the saddle-shaped nose due to a perforated nasal septum. This causes a dip halfway down the nose.

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

In the kidneys, it can cause rapidly progressing glomerulonephritis.

Question 16

Polyarteritis Nodosa

Answer 16

Polyarteritis nodosa (PAN) is a medium vessel vasculitis. It is most associated with hepatitis B but can also occur without a clear cause or with hepatitis C and HIV.

It affects the medium sized vessels in locations such as the skin, gastrointestinal tract, kidneys and heart. This can cause renal impairment, strokes and myocardial infarction.

It is associated with a rash called livedo reticularis. This is a mottled, purplish, lace like rash.

Question 17

Kawasaki Disease

Answer 17

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 18

Takayasu’s arteritis

Answer 18

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.