Wegener’s granulomatosis

This is an inflammation of the blood vessels which affects many internal organs of the body which include the lungs, heart and kidneys.

The throat is also affected and becomes red, swollen and inflamed.

This rare but serious condition is potentially fatal if left untreated.

Causes of Wegener’s granulomatosis

No-one is certain as to the exact cause of this disease although it is thought to be caused by a ‘fault’ within the immune system. This leads to the immune system turning upon its host.

This fault within the immune system or an autoimmune disease is likely to have developed within someone who is at high risk of doing so. They may have contracted a bacterial or viral infection which triggers this autoimmune disease.

There is very little known about this condition which means that it is impossible to prevent it from occurring in the first place.

Symptoms of Wegener’s granulomatosis

These affect many areas of the body which include:

• Sore throat
• Rhinitis (inflammation of the nasal airways)
• Conjunctivitis
• Glomerulonephritis (inflammation of the kidneys)
• Sinusitis
• Arthritis
• Chronic ear infections
• Narrowing of the trachea

These are just some of the numerous symptoms caused by this disease.

How is this diagnosed?

There is a set of criteria to help with confirming a diagnosis which includes ulcers caused by inflammation of the nose or mouth; nodules on the lungs; blood in the urine and inflammation of the blood vessels –known as ‘vasculitis’.

Several methods are used to confirm or reject this diagnosis. Chest X-rays, biopsy (blood vessels) and urine tests are combined in order to help with this.

This condition is often only diagnosed when someone has been suffering with these symptoms for a long period of time.

Treatment for Wegener’s granulomatosis

This involves immunosuppressant drugs such as corticosteroids and cyclophosphamide which help to reduce any inflammation. Plasma exchange is another option which involves the removal, treatment and then re-infusion of the plasma back into the body.

Some people develop subglottic stenosis – a condition in which the airway below the vocal cords is too narrow which causes breathing difficulties. In these cases a tracheostomy is required.

The main risk with Wegener’s granulomatosis is kidney failure so it is important that the person be monitored on a regular basis.