Granulomatosis with polyangiitis (GPA)
Granulomatosis with polyangiitis (GPA) is a rare condition where blood vessels become inflamed, mainly in the ears, nose, sinuses, kidneys and lungs. It can be serious if not treated but medicines can help manage the symptoms.
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Symptoms of granulomatosis with polyangiitis (GPA) Important See a GP if: Tests for granulomatosis with polyangiitis (GPA)Treatments for granulomatosis with polyangiitis (GPA)Stage 1: Bringing the condition under controlStage 2: Keeping the condition under controlTreating symptoms if they come backTreating severe casesRecording information about you and your condition -
Medicines commonly prescribed for Granulomatosis with polyangiitis (GPA)
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: ORAL SOLUTION/SUSPENSION DROPS, 13.3 mg prednisolone estragelate/mlActive substance: prednisoloneManufacturer: Laboratorios Sonphar S.L.Prescription requiredDosage form: TABLET, 75 mgActive substance: azathioprineManufacturer: Dr. Falk Pharma GmbhPrescription requiredDosage form: ORAL SOLUTION/SUSPENSION, 4 mg/mlActive substance: prednisoloneManufacturer: Laboratorio Aldo Union S.L.Prescription required
Granulomatosis with polyangiitis (GPA) is a rare condition where blood vessels become inflamed, mainly in the ears, nose, sinuses, kidneys and lungs. It can be serious if not treated but medicines can help manage the symptoms.
GPA was previously called Wegener's granulomatosis.
Symptoms of granulomatosis with polyangiitis (GPA)
Granulomatosis with polyangiitis (GPA) is a type of vasculitis, which is an inflammation of the blood vessels.
Symptoms vary between people, and can be mild or severe. In some people, they come on slowly over many months, and in others they appear suddenly.
General symptoms may be mild and start before symptoms in specific parts of the body. They can include:
- high temperature
- night sweats
- feeling weak and tired
- joint pain
- loss of appetite
- weight loss
Other symptoms depend on which parts of your body are affected, so you may not have all the symptoms.
Symptoms that may affect specific parts of the body
Body part Possible symptoms
| Ears, nose and sinuses | Ear infection, hearing loss, blocked or runny nose, crusting of blood and mucus in the nose, nosebleeds, sinus pain
| Eyes | Conjunctivitis, swollen eyelids, double vision, blurred vision
| Lungs | A cough that does not go away, coughing up blood, shortness of breath, chest pain, wheezing
| Kidneys | High blood pressure, swelling in the legs, kidney failure
| Skin | Rashes, ulcers, small spots
| Stomach | Tummy pain, diarrhoea, blood in poo
Important
Some of these symptoms are very common and can be caused by other conditions.
Having the symptoms does not definitely mean you have GPA, but it's important to get checked by a GP.
See a GP if:
- you have symptoms of granulomatosis with polyangiitis (GPA)
If you have been diagnosed with GPA, and have new or returning symptoms, see the specialist treating you.
Tests for granulomatosis with polyangiitis (GPA)
There's no single test for granulomatosis with polyangiitis (GPA) and it can be difficult to diagnose.
A GP will ask about your symptoms and may examine the affected part of your body. If they think you could have GPA they'll refer you to a specialist.
You'll have tests such as:
- a blood test to see if you have an antibody called ANCA (antineutrophil cytoplasmic antibodies), which may be a sign of GPA
- a biopsy, where a small sample of tissue from any affected body parts is sent for testing
- an X-ray, CT scan or MRI scan of any affected body parts
- a urine (pee) test if you have symptoms affecting your kidneys
Treatments for granulomatosis with polyangiitis (GPA)
You'll be treated by a specialist if you have granulomatosis with polyangiitis (GPA).
Treatment depends on how severe it is and the body parts affected.
There are 2 stages of treatment – bringing the condition under control and keeping it under control.
Stage 1: Bringing the condition under control
If you have mild GPA, you may be given an anti-inflammatory medicine called a steroid to control your symptoms. You may also have an immunosuppressant medicine such as methotrexate.
If you have severe GPA, you may be given a biological medicine such as rituximab or avacopan, or both, along with steroids.
You may also be given a type of chemotherapy medicine, such as cyclophosphamide, to bring it under control.
Stage 2: Keeping the condition under control
Once the condition is under control, treatment aims to stop your symptoms coming back.
You may have lower doses of your medicines or take a different type of medicine.
Treating symptoms if they come back
If your symptoms come back or you get new symptoms (a relapse), your treatment may be changed or restarted.
Treating severe cases
In very severe cases that affect the kidneys, you may need dialysis or a kidney transplant.
You may also need surgery to the affected body part if you have complications from GPA, for example reconstructive surgery if the bridge of the nose has been damaged.
Recording information about you and your condition
It's used to help scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.
Online doctors for Granulomatosis with polyangiitis (GPA)
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