Wegener's granulomatosis
HowGPA istreated
The aim of treatment for GPA is firstly to bring the condition under control (known as inducing remission), and then try to prevent the condition relapsing (known as maintaining remission).
For most people, this can be achieved using a number of different medications, which may need to be taken for a number of years. Medication administration is supervised through specialist centres in close liaison with your GP and is tailored to each individual patient.
Inducing remission
To begin with, you will usually need high doses of medication to dampen down your immune system and bring the inflammation under control.
Typically, you will be given a steroid medication called prednisolone to reduce the inflammation in addition to a medication called cyclophosphamide, which weakens your immune system.
Steroid medication is either given intravenously alongside this, or is taken by mouth (orally) on a daily basis.
Cyclophosphamide is normally given intravenously (directly into a vein) every two to three weeks.
In milder cases, an alternative oral immune-suppressing medication called methotrexate may be given instead of cyclophosphamide.
This treatment will continue with your steroid dose gradually reduced over time until you achieve remission, which in most people takes around three to six months.
Cyclophosphamide and steroids are powerful medications, so make sure you discuss the possible side effects with your doctor. This medication works by destroying certain cells produced by the immune system that are thought to play a role in the inflammation of the blood vessels.
In particularly severe cases affecting the kidneys, a procedure called plasma exchange (plasmapheresis) may be recommended.
During this procedure, you will be connected to a machine that removes some of your blood. A blood component called plasma will then be separated from the blood cells and removed, before the remainingblood cells are returned to your body. This process can remove some of the antibodies thought to contribute to the inflammation in GPA.
Once the disease is under control, some people may also need surgical treatment. For example, surgery may be needed to correct any nose deformities, narrowing of the airways or obstruction of the tear ducts that can occur as a result of the tissue damage caused by GPA.
If damage to the kidneys means they are no longer able to work properly, a kidney transplant may be needed.
Medically Reviewed by a doctor on 21 Dec 2018
Introduction
Granulomatosis with polyangiitis (GPA) is a rare disease which causes the walls of the blood vessels to become inflamed (swollen).
Signs and symptoms
The inflammation of the blood vessels (known as vasculitis) caused by GPA mainly affects the nose, sinuses, ears, lungs and kidneys, although other organs can be affected too, including the brain, ski
When to seek medical advice
If you have any of the above symptoms that are persistent or troublesome see your GP. They can arrange appropriate tests if they suspect GPA is the cause and, if appropriate, arrange referral to a spe
What causes GPA?
The exact cause of GPA is unknown. It is thought that GPA is likely to be an autoimmune disease, which means the body's immune system mistakenly attacks its own tissues. As with other autoimmune diso
Who is affected
Around 500 men and women are diagnosed with GPA each year in the UK. Symptoms can start at any age, including in childhood, although the condition usually first appears in middle-aged or elderly peop
How GPA is diagnosed
Your doctor may suspect GPA if you feel unwell, have a persistent fever and have lost weight, and one or more of the following areas of your body is affected: your nose, ears and sinuses for examp
HowGPA istreated
The aim of treatment for GPA is firstly to bring the condition under control (known as inducing remission), and then try to prevent the condition relapsing (known as maintaining remission). For most
Outlook
Although GPA can be life-threatening, early diagnosis and modern treatments can significantly improve the outlook. Most people are able to achieve remission with medical treatment, although about hal
Information about you
If you have GPA, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS). This helps scientists look for better ways
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