Living with granulomatosis with polyangiitis living

Overview

Granulomatosis with polyangiitis, or GPA, is a rare disease in which the immune system mistakenly attacks small blood vessels. This causes swelling and damage, most often in the nose, sinuses, lungs, and kidneys. It used to be called Wegener's granulomatosis. With early treatment, most people can manage the condition and live well.

No, GPA is rare. It affects about 1 to 3 people per 100,000 in the UK and other countries.

GPA can happen at any age, but it is most often diagnosed in people between 40 and 60. It affects both men and women, and can occur in children, though this is much less common.

Symptoms

Common symptoms

• Persistent runny nose or sinus infections
• Nosebleeds or crusting inside the nose
• Cough, sometimes with blood
• Shortness of breath or wheezing
• Feeling very tired or unwell
• Fever and night sweats
• Joint aches and pains
• Skin rashes, lumps, or sores
• Blood in urine or foamy urine
• Eye redness, pain, or vision problems

Symptoms in children

• Children with GPA may have the same symptoms as adults, including sinus problems, cough, and joint pain.
• They may also have poor growth, weight loss, or fatigue beyond normal childhood tiredness.
• Skin rashes and kidney inflammation are also possible in children.

Symptoms in older adults

• In older adults, symptoms may be less typical and can include fatigue, weight loss, and mild kidney problems.
• Symptoms such as cough or joint pain can be mistaken for other age-related conditions, so diagnosis may be delayed.
• Older adults may have more severe disease at diagnosis, so it is important not to ignore persistent symptoms.

Causes

Main causes

• The exact cause of GPA is unknown, but it is an autoimmune disease — the body's immune system attacks its own tissues.
• The condition causes inflammation in small blood vessels, which limits blood flow and damages organs.
• Both genetic factors and environmental triggers, such as infections or certain exposures, may play a role.

Risk factors

• Having a family history of autoimmune diseases may increase risk slightly.
• Infections, especially of the nose or sinuses, might trigger the disease in some people.
• Smoking appears to increase the risk and may make lung involvement worse.

When to see a doctor

See a doctor urgently if:

• If you have any of the emergency symptoms listed above, call your local emergency number or go to an emergency department right away.
• See a doctor the same day if you have a high fever, are coughing up blood, or notice major swelling or shortness of breath.

Book a routine appointment if:

• Make an appointment with your GP if you have a runny nose or sinus problems that last longer than a few weeks.
• See your doctor if you feel very tired, have joint pains, or are losing weight without trying.
• Get checked if you notice blood in your urine or have a change in how much you urinate.

Diagnosis

Diagnosing GPA involves a combination of medical history, physical examination, and several tests. Because the disease is rare and affects many parts of the body, doctors often work as a team, including rheumatologists, kidney specialists, and lung specialists.

Tests that may be done

• Blood tests to check for inflammation and special antibodies seen in GPA
• Urine tests to look for blood or protein, which can signal kidney damage
• Chest X-ray or CT scan to check lung involvement
• Biopsy — taking a small sample of tissue from the nose, lungs, or kidney — to confirm the diagnosis
• Sometimes a bronchoscopy, which uses a flexible tube to look inside the airways

What to expect at your appointment

If your doctor suspects GPA, you will likely be referred to a specialist for further tests. It may take several weeks to confirm the diagnosis. This can be a worrying time, but getting the right diagnosis is important so treatment can start as soon as possible.

Treatment

Treatment for GPA aims to control inflammation, stop the immune system from damaging the body, and keep the disease in remission. Most people need a combination of medicines and long-term follow-up. Treatment is usually led by a specialist team.

Self-care at home

• Take your medications exactly as prescribed, even if you feel better.
• Protect yourself from infections by washing hands often and avoiding people who are sick.
• Get plenty of rest when you feel tired, and pace yourself during busy times.
• If you smoke, ask your doctor about stopping — smoking can worsen lung damage.
• Keep all your follow-up appointments for blood tests and scans.

Medical treatments

Medical treatment usually starts with strong anti-inflammatory medicines (corticosteroids) and other medicines that quiet the immune system. Some people may need a treatment called plasma exchange, which filters antibodies from the blood, if the disease is severe. In recent years, newer immune-modifying medicines have made treatment more personalised. Your specialist will choose the best plan for you.

When is surgery considered?

Surgery is rarely needed for GPA itself. In some cases, it may be used to repair damage caused by the disease, such as a narrowed windpipe, a hole in the nasal septum, or complications from kidney failure. Most people do not need surgery.

Living with this condition

Living with GPA means learning to manage a long-term condition. Many people go through periods of remission where symptoms are minimal, and periods of flare-ups. Following your treatment plan, monitoring your symptoms, and keeping in touch with your care team are the most important parts of daily life.

Lifestyle tips

• Choose a balanced diet with fruits, vegetables, lean proteins, and whole grains.
• Stay physically active with gentle activities like walking or swimming, as your energy allows.
• Get enough sleep and try stress-reducing activities like deep breathing or meditation.
• Avoid smoking and limit alcohol to protect your lungs and kidneys.
• Stay up to date with vaccinations, but talk to your doctor first because some live vaccines are not safe with certain treatments.

Diet and exercise

A healthy diet helps support your immune system and bones, especially if you take steroids. Exercise, such as walking or stretching, can help maintain strength and reduce fatigue. If you have kidney problems, your doctor may refer you to a dietitian for advice on potassium and salt intake.

Mental health and emotional wellbeing

A chronic illness like GPA can be emotionally challenging. It is normal to feel anxious, stressed, or sad at times. Talking to a counsellor, joining a support group, or simply sharing your feelings with trusted people can help. Your care team can also provide guidance about mental health resources.

Prevention

There is no known way to prevent GPA, since the cause is not fully understood. However, early recognition and treatment can prevent serious organ damage and improve your outlook.

Vaccines

People with GPA and their close contacts should make sure routine vaccines are up to date, such as flu, COVID-19, and pneumonia vaccines. But some vaccines, especially live ones, are not recommended during certain treatments. Always ask your specialist or pharmacist for advice.

Screening programmes

There is no routine screening test for GPA in the general population. If you have a family history of vasculitis or autoimmune disease and are experiencing concerning symptoms, talk to your doctor.

Complications

If left untreated

• Kidney damage that may progress to kidney failure
• Permanent lung damage, including scarring or bleeding in the lungs
• Nerve damage causing weakness, tingling, or pain
• Damage to the eyes, leading to vision loss
• Hearing loss or damage to the sinuses and nasal passages
• Increased risk of heart attacks or strokes

Long-term outlook

With modern treatment, most people with GPA improve significantly and many reach remission. Remission means the disease is not active, and symptoms may disappear for months or years. It is a lifelong condition that needs regular monitoring, but many people continue to work, spend time with family, and pursue the activities they enjoy. Success depends on early treatment, sticking to your plan, and working closely with your healthcare team.

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