Medical knowledge, Patient Education
Granulomatosis With Polyangiitis
Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare disease of the blood vessels. It causes inflammation of small-sized and medium-sized blood vessels in many parts of the body. This inflammation can interfere with the function of organs that are supplied by the blood vessels. Organs that are commonly affected by this condition include the kidneys, lungs, nose, ears, skin, and eyes. The nervous system is also commonly affected.
This condition can range from mild to severe, and it can develop gradually or very quickly (be acute). In severe cases, the condition can cause serious damage to organs such as the lungs or kidneys. Early treatment can help to prevent damage to the organs that are involved.
What are the causes?
The cause of this condition is not known. It occurs when the body’s defense system (immune system) mistakenly attacks the body’s own blood vessels, which causes inflammation and damage. This condition is an autoimmune disease.
What increases the risk?
You are more likely to develop this condition if you are between the ages of 55 and 70.
What are the signs or symptoms?
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General symptoms include:
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Severe tiredness (fatigue) and weakness.
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Decreased appetite.
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Unintended weight loss.
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Fever or sweats.
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Joint pain or swelling.
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Muscle pain.
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Symptoms that involve the nose include:
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Nose or face pain.
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Runny nose.
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Crusts or sores in the nose.
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Nosebleeds.
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Symptoms that involve the airway and lungs include:
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Cough.
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Chest pain.
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Change in voice.
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Wheezing, or making high-pitched whistling sounds when you breathe, most often when you breathe out.
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Shortness of breath.
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Coughing up blood.
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Symptoms that involve the eyes include:
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Eye pain.
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Red eyes.
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Vision problems.
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Symptoms that involve the ears include:
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Hearing problems.
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Symptoms that involve the nervous system include:
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Headache.
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Loss of feeling and nerve pain (neuropathy).
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Seizures.
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Symptoms that involve the kidneys include:
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Blood in the urine.
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Decreased kidney function.
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Many other parts of the body can be involved.
How is this diagnosed?
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A physical exam.
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Your medical history.
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Blood tests.
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Urine tests.
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Imaging tests, such as X-rays or a CT scan.
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A biopsy. A small tissue sample is taken from the nasal passages, lung, kidney, skin, or other affected areas for examination in a lab.
You may be referred to a specialist. This condition can be confused with a number of other rare diseases.
How is this treated?
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Steroid medicines, such as prednisone.
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Other medicines that prevent or slow down your immune system from causing further damage to your blood vessels.
You may also be given antibiotic medicines to prevent infection and to help prevent symptoms from returning.
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You may be given medicine directly into a vein through an IV.
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You may have a type of blood plasma exchange (plasmapheresis) to remove immune system cells that are attacking your blood vessels.
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If your kidneys are badly damaged, a kidney transplant may be recommended.
Because it is common for symptoms to come back again (relapse), treatments may need to be adjusted.
Follow these instructions at home:
Medicines
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Take over-the-counter and prescription medicines only as told by your health care provider.
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If you were prescribed an antibiotic medicine, take it as told by your health care provider. Do not stop using the antibiotic even if you start to feel better.
Eating and drinking
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Try to eat regular, healthy meals. This includes plenty of fresh fruits and vegetables, whole grains, low-fat (lean) protein, and low-fat dairy products.
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Drink enough fluid to keep your urine pale yellow.
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Take vitamins and supplements as told by your health care provider or dietitian.
General instructions
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Follow instructions from your health care provider about testing for blood in your urine.
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Keep your vaccinations up to date. Get the flu (influenza) vaccine every year.
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Do not use any products that contain nicotine or tobacco. These products include cigarettes, chewing tobacco, and vaping devices, such as e-cigarettes. If you need help quitting, ask your health care provider.
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Keep all follow-up visits. This is important.
Where to find more information
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National Organization for Rare Disorders: rarediseases.org
Contact a health care provider if:
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Your symptoms do not improve with treatment.
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You develop any new symptoms or problems.
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You have a fever.
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You have nausea or diarrhea.
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You develop a rash or an infection.
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You have a sore throat, white patches in your mouth, or difficulty swallowing.
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You have severe fatigue.
Get help right away if:
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You have chest pain.
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You feel short of breath.
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You feel very light-headed, or you pass out.
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You have:
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Pain, swelling, or redness anywhere in your legs.
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Decreased urination or blood in your urine.
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Uncontrollable bleeding, such as a nosebleed that will not stop.
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Sudden loss of vision or hearing.
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Do not wait to see if the symptoms will go away.
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Do not drive yourself to the hospital.
Summary
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Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare disease of the blood vessels.
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GPA causes inflammation of small-sized and medium-sized blood vessels in many parts of the body.
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This condition can range from mild to severe, and it can develop gradually or very quickly (be acute).
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Symptoms can vary depending on which part of the body is involved.
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This condition is treated with medicines. Because relapses are common, treatments may need to be adjusted.
This information is not intended to replace advice given to you by your health care provider. Make sure you discuss any questions you have with your health care provider.
