What is Granulomatosis With Polyangiitis (GPA)
Granulomatosis with polyangiitis , also known as Wegener 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 (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 a person’s defense system (immune system) mistakenly attacks the body’s own blood vessels, which causes inflammation and damage. This makes GPA an autoimmune disease.
What increases the risk?
This condition is more likely to develop in:
- People who are older than 40 years of age.
- People who are of Caucasian ethnicity.
What are the signs or symptoms?
Symptoms can vary depending on which part of the body is involved.
- General:
- Severe tiredness and weakness.
- Decreased appetite.
- Unintended weight loss.
- Fever or sweats.
- Joint pain or swelling.
- Muscle pain.
- Nose:
- Nose or face pain.
- Runny nose.
- Crusts or sores in the nose.
- Nosebleeds.
- Airway
and lungs:
- Cough.
- Chest pain.
- Change in voice.
- Wheezing.
- Shortness of breath.
- Coughing up blood.
- Eyes:
- Eye pain.
- Red eyes.
- Vision problems.
- Ears:
- Hearing problems.
Many other parts of the body can be involved.
How is this diagnosed?
This condition may be diagnosed with:
- A physical exam and medical history.
- Blood tests.
- Urine tests.
- Imaging tests, such as X-rays or a CT scan.
- 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?
Aggressive treatment is important for this condition and can lead to remission. GPA is usually treated with:
- Steroid medicines, such as prednisone.
- 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 the disease from returning.
In severe cases, you may be given medicine directly into a vein through an IV tube. A type of blood transfusion (plasmapheresis) may also be done to remove immune system cells that are attacking your blood vessels. If the kidneys are badly damaged, a kidney transplant may be recommended. Because relapses are common, treatments may need to be adjusted.
Follow these instructions at home:
- Take over-the-counter and prescription medicines only as told by your health care provider.
- If you were prescribed an antibiotic medicine, take it as told by your health care provider. Do notstop taking the antibiotic even if you start to feel better.
- Follow instructions from your health care provider about testing for blood in your urine.
- Take vitamins and supplements as told by your health care provider or dietitian.
- Follow instructions from your health care provider about diet. Try to eat regular, healthy meals, even though some of your treatments might affect your appetite.
- Keep all follow-up visits as told by your health care provider. This is important.
Contact a health care provider if:
- Your symptoms do not improve in the time expected.
- You develop any new symptoms or problems.
- You have a fever.
- You have nausea or diarrhea.
- You develop a rash.
- You have a sore throat, white patches in your mouth, or difficulty swallowing.
- You have severe fatigue.
- You develop an infection.
Get help right away if:
- You have chest pain.
- You feel short of breath.
- You feel very light-headed, or you pass out.
- You have pain, swelling, or redness anywhere in your legs.
- You have blood in your urine.
- You have uncontrollable bleeding, such as a nosebleed that will not stop.
- You have sudden loss of vision or hearing.
- You have decreased urination.
