Vasculitis

 

What is Vasculitis?

Vasculitis means inflammation in the blood vessels. It is a broad name for a group of conditions that affect different sizes of blood vessel.

 

Blood vessels transport blood around the body – arteries take blood from the heart to the organs and veins transport blood back to the heart. Arteries and veins around the heart start off large, and as they move further away, become smaller. The smallest vessels are called capillaries; these pass oxygen and nutrients to muscles and organs.

 

The immune system usually produces inflammation in response to injury or infection.  However, in vasculitis, inflammation is targeted at healthy blood vessels, causing damage to the vessel wall which narrows the channel and leads to reduced blood supply to tissues.  This can result in thinning and ballooning of the walls causing aneurysms. The overactivation of the immune system against normal blood vessels suggests that most forms of vasculitis are autoimmune diseases.

 

There are many types of vasculitis; the easiest way to classify them is by the size of the blood vessel they affect.

 

Subtypes of vasculitis

Large arteries

• Giant Cell Arteritis (GCA)
• Takayasu arteritis

Medium arteries

• Polyarteritis nodosa (PAN)
• Kawasaki disease

Small vessels and medium arteries

• ANCA associated vasculitis- EGPA/ GPA
• Vasculitis secondary to RA/ SLE/ Sjogrens/ Drugs

Small vessels

• Henoch- Schonlein purpura IgA vasculitis
• Drugs
• Cryoglobulinaemia/ Hep C
• Cutanous leucocytoclastic angiitis/ infection

 

Causes of vasculitis

The cause of vasculitis is unknown, but evidence suggests it is caused by a number of factors such as genetics and environmental factors.

Some genes have been linked to increased risk of developing vasculitis, although it isn’t directly inherited and other triggers are likely needed.

There are some well-known environmental triggers for vasculitis. In these cases, we would call these secondary vasculitides.  Viral and bacterial infections in particular are known to cause certain types of vasculitis. Smoking and UV light exposure are also implicated. Certain medications are also linked to developing vasculitis, these usually only cause skin rashes and resolve on withdrawing of the drug.

Rarely a small number of cancers have been known to cause vasculitis.

 

Symptoms of vasculitis

Different types of vasculitis often present in different ways, however there are some common symptoms of all:

General symptoms

• Fever
• Weight loss
• Fatigue- extreme tiredness
• Myalgia- muscle aches, particularly after exercise, improving at rest
• Arthralgia- joint aches
• Arthritis- swollen, tender and stiff joints

 

Specific areas can be involved in certain types of vasculitis:

 

Skin lesions

• Palpable purpura
  • Purple spots that don’t blanch (go white) on pressing, these can group to form larger areas and bruises
  • Often present in legs and feet
• Ulcers
• Raynaud’s Phenomenon
• Gangrene can occur in the finger tips and toes
• Mouth ulcers

Eyes

• Red inflamed eyes, difficulty looking at bright lights

Lung and airway involvement

• Crusting, nosebleeds and nose deformities
• Asthma
• Lung nodules and airway bleeding
• Commoner in ANCA associated vasculitis

Heart involvement

• Pericarditis- inflammation in the heart lining causing chest pain
• Myocarditis- inflammation in the heart muscle
• Abnormal heart rhythms
• Heart attacks

Kidney involvement

• Leaking blood and protein into the urine (glomerulonephritis)

Gut involvement

• Abdominal pain, bleeding

Nervous system

• Pins and needles, numbness, weakness in the arms and legs
• Headaches
• Meningitis
• Strokes

 

How do we diagnose vasculitis?

Diagnosis is made based on history, examination and investigations.

 

Blood tests:

• Full blood count
• Liver and kidney function
• Inflammation markers- ESR and CRP
• Autoimmune screen
  • ANA, rheumatoid factor
  • Immunoglobulins
  • Complements
  • ANCA- antibodies specific to certain group of vasculitis

Urine dip- to check for protein and blood

Imaging

• X rays- of chest and joints
• Ultrasound of joints, abdominal organs and temporal arteries
• CT and MRI scans can show which blood vessels are inflamed

Heart investigations

• Electrocardiogram (ECG) heart tracing
• Echocardiogram (ECHO)- ultrasound imaging of heart structure

Nerve tests

• Nerve conduction studies can show if a specific nerve is involved

Biopsies

• Samples of tissue can be sent to the laboratory and looked at under a microscopy by specialists to help with the diagnosis.
• These can be from; skin, blood vessel walls, nerves, lesions in the nose or airway and kidney.

 

Types of Vasculitis

Giant Cell Arteritis (GCA)

• Vasculitis affecting the large arteries of the head and neck, in particular the temporal arteries.
• Common symptoms include headache, muscle aches and pains (PMR), temporal tenderness.
• Rarer more serious side effects include changes in vision resulting in blindness if left untreated and jaw pain on chewing.
• More information can be found on our dedicated pages for GCA.

Takayasu Arteritis

• Vasculitis of the branches of the aorta from the heart, symptoms are therefore linked to the branches below:
  • Subclavian arteries supplying the arms
  • Carotid arteries supplying the brain and head
  • Renal arteries to the kidneys
  • Coronary arteries supplying the heart muscle
• It more commonly affects females, those aged less that 40 years and is rare in the UK. It is much more prevalent in Asia, particularly Japan
• Classical signs can be found:
  • Pulses in the arms and legs can be absent
  • There can be difference in blood pressure between the arms

Polyarteritis Nodosa (PAN)

• This is a rare type of vasculitis that commonly affects the nerves, skin, limbs and interstines.
• It occurs in children and adults and has been linked to hepatitis B infections.
• A classical skin rash could be present called livedo reticularis, which appears as a mottling over the arms, legs and torso.

ANCA associated vasculitis

• 3 types of small vessel vasculitis are included as they are associated with positive blood antibodies to ANCA
  • Microscopic polyangiitis (MPA)
  • Granulomatosis with polyangiitis (GPA)
  • Eosinophilic granulomatosis with polyangiitis (EGPA)
• MPA is a rare vasculitis that primarily affects the kidneys and lungs
  • It is more prevalent in Caucasians
  • It is associated with MPO pANCA blood test
• GPA was formerly known as Wegener’s granulomatosis
  • It is rare with only a handful of new cases each year in the UK
  • It usually affects the ears, nose, eyes and throat as well as the kidneys.
  • It is associated with a PR3 cANCA blood test
• EGPA was formerly known as Churg-Strauss syndrome
  • Classical symptoms include asthma and allergic rhinitis
  • Blood tests show raised Eosinophils (a type of white blood cells)
  • It is associated with a MPO pANCA blood test
  • It more commonly affects the heart muscle and nerves

IgA Vasculitis

• A small vessel vasculitis that often follows a chest infection
• It occurs in adults, but is more common in children
• The first symptom is usually a skin rash of purpura that commonly occurs over the buttock and lower limbs. Arthritis can occur in the larger joints. Rarely the gut can be involved leading to blood stools. The kidneys can also be involved.
• The disease is usually self-limiting, but in a small number of cases relapses of symptoms occur.

Cryoglobulinaemia

• This small vessel vasculitis occurs when proteins called cryoglobulins are produced that stick together in the cold. This means that they usually block blood vessels in the peripheries such as the hands and feet resulting in a purple rash with ulcers.
• There are 3 subtypes depending on which immunoglobulin (immune protein) is produced.
• They are more often seen associated with cancers of the blood and viral infections such as Hepatitis C.

 

Treatment 

Management of vasculitis is tailored to the type of vasculitis and the blood vessels involved. The aim of treatment is to prevent any complications and permanent damage to organs such as the heart, kidneys and brain.

Treatment is based on quickly inducing remission (control) of the inflammation and then maintenance treatments are used to prevent any further flares of the disease.

 

Remission induction is based on the use of high dose steroids and in some severe life threatening cases of vasculitis, other medications to suppress the immune system are used such as DMARDs and biological therapies.

 

Maintenance therapies include long-term use of DMARDs.

In some patients, once they have been stable with no flares of the disease, treatment can be slowly withdrawn.

 

If a clear cause is found to have caused the vasculitis such as a hepatitis infection or drug, then management of these is important.

 

Rarely if the damage to the blood vessels is severe causing aneurysms, surgery may be indicated.

 

Useful links

Versus Arthritis: http://www.versusarthritis.org

Vasculitis Scotland Support network: http://vasculitis-scotland.org

Vasculitis UK: https://www.vasculitis.org.uk