Wegener's granulomatosis
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 half of people with GPA will have a relapse at some point. This usually happens within a few years of stopping treatment.
If you do have a relapse, you will usually need to start taking medication again or increase the doses of any medication you are already taking.
Most people with GPA are able to lead normal lives, although in addition to taking medication regularly you will need to have regular check-ups to monitor your progress and check for signs of a relapse.
Complications, if they occur, are often the result of a delay in diagnosis and a lack of treatment. If left untreated, people with GPA may develop severe inflammation in the respiratory tract and kidneys, which can eventually lead to life-threatening lung and kidney failure.
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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