Vasculitis

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.

 

Types of Vasculitis Affecting The Small Vessels

  • Henoch-Schonlein purpura
  • Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
  • Microscopic polyangiitis
  • Granulomatosis with polyangiitis (Wegener’s granulomatosis)

 

Types of Vasculitis Affecting The Medium Sized Vessels

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

 

Types of Vasculitis Affecting The Large Vessels

  • Giant cell arteritis
  • Takayasu’s arteritis

 

Presentation

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

  • 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

 

There are some features that are more specific to individual types and these are discussed below.

 

Tests

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): Wegener’s granulomatosis

 

Management

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.

 

Henoch-Schonlein purpura

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.

The four classic features are purpura (100%), joint pain (75%), abdominal pain (50%) and renal involvement (50%). HSP affects the kidneys in about 50% of patients, causing an IgA nephritis.

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.

 

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

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.

 

Microscopic polyangiitis

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.

 

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

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) 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 xray may show consolidation and it may be misdiagnosed as pneumonia.

In the kidneys it can cause a rapidly progressing glomerulonephritis.

Polyarteritis Nodosa

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.

 

Kawasaki Disease

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.

 

Takayasu’s arteritis

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.

 

Last updated 2019
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