Wegener’s Granulomatosis

What is Wegener's granulomatosis?

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Wegener’s granulomatosis is a type of vasculitis, or disorder that produces inflammation in the blood vessels. Wegener’s granulomatosis damages and inflames blood vessels in several organs, including the lungs, sinuses, and kidneys. Inflammation restricts blood flow and prevents oxygen from traveling to your tissues and organs, which ultimately impacts how well they can function. Granulomas, or inflamed tissue lumps, grow around blood vessels and damage organs.

What causes Wegener's granulomatosis?

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Wegener’s granulomatosis is an autoimmune disease, which means that the body’s immune system turns against its own healthy tissues. This condition causes the immune system to attack blood vessels, specifically.

Experts do not know what causes Wegener’s granulomatosis. Genetics do not play a role, as Wegener’s granulomatosis does not usually run in families. Infections might cause this condition. Bacteria and viruses cause the body to send out cells that can create inflammation. That response could harm otherwise healthy tissues. However, Wegener’s granulomatosis is not connected to any fungus, bacteria, or virus.

What are symptoms of Wegener's granulomatosis?

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Early stages of Wegener’s granulomatosis do not usually present symptoms. In more advanced stages, this condition targets the lungs, sinuses, and kidneys. This condition can progress quickly once symptoms appear.

Symptoms of Wegener’s granulomatosis can include:

  • Joint pain
  • Skin sores, rashes, or bruising
  • Coughing (sometimes with bloody phlegm)
  • Bloody urine
  • Fever
  • Weight loss
  • Shortness of breath
  • Drainage from the nose
  • Sinus infections
  • Nosebleeds
  • Fatigue
  • Numbness in the toes, fingers, or limbs
  • Eye pain, burning, or redness
  • Vision problems
  • Ear inflammation
  • Hearing problems

Are there any risk factors or groups for Wegener's granulomatosis?

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The cause of Wegener’s granulomatosis is unknown. As such, it is difficult to identify risk factors. You can develop Wegener’s granulomatosis at any age, but the condition most often occurs between the ages of 40 and 65.

How is Wegener's granulomatosis diagnosed?

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First, you will discuss your symptoms and medical history with your doctor. Then, your doctor will determine which tests to perform in order to make a diagnosis.

Urine and Blood Tests

  • C-reactive protein and erythrocyte sedimentation rate (sed rate): These blood tests help pinpoint inflammation within the body.
  • Antineutrophil cytoplasmic antibody test: This blood test searches for antibodies that most people with Wegener’s granulomatosis have. Doctors cannot use this test to definitively determine whether you have Wegener’s granulomatosis because approximately 20% of people with the condition actually have a negative antineutrophil cytoplasmic antibody test.
  • Complete blood count: This blood test measures your body’s blood cell count. Low numbers could indicate that you have anemia, which commonly occurs when Wegener’s granulomatosis affects the kidneys.

Imaging Tests

  • X-rays: A chest X-ray will use trace amounts of radiation to take images of your blood vessels and lungs.
  • CT scan: A CT scan uses rotating X-ray machines and computers to take more in-depth pictures of the affected areas.
  • MRI scan: An MRI uses radio waves and magnets to create cross-sectional pictures of the affected areas.


Your doctor must use a biopsy to make a Wegener’s granulomatosis diagnosis. A biopsy is a surgical procedure in which a doctor will take a small tissue sample from an affected area, like your kidney or lung, and give it to a lab for diagnostic confirmation. At the lab, a technician will determine whether the tissue sample contains evidence of Wegener’s granulomatosis. If urine, blood, and imaging test results are inconclusive or abnormal, then your doctor might order a biopsy to help make a diagnosis.

How is Wegener's granulomatosis treated?

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Your doctor might recommend a few different strategies to treat Wegener’s granulomatosis. You might take certain medications indefinitely to keep the disease from returning. The two most common treatments for Wegener’s granulomatosis are medications and plasma exchange.


  • Corticosteroids: Corticosteroids are an anti-inflammatory drug, commonly used to treat lupus, rheumatoid arthritis, and vasculitis.
  • Chemotherapy drug rituximab: This drug is usually used to treat cancer, like non-Hodgkin’s lymphoma and chronic lymphocytic leukemia. However, rituximab can also treat rheumatoid arthritis and vasculitis by reducing joint pain and swelling.
  • Immunosuppressant drugs: Immunosuppressant drugs reduce the immune system’s strength. They treat autoimmune diseases, like Wegener’s granulomatosis.

Sometimes, doctors will recommend combining drugs to reduce inflammation. A common combination would be cyclophosphamide and prednisone. Many people with Wegener’s granulomatosis recover using this medication combination.

Mild cases of Wegener’s granulomatosis might resolve using methotrexate and prednisone. Methotrexate is an immunosuppressant and prednisone is a corticosteroid. This combination has fewer side effects than cyclophosphamide and prednisone.

Side effects from these drugs can include reducing your body’s ability to fight infection and weakening your bones. Some medications also cause weight gain. Your doctor will keep tabs on whether these side effects develop and help you address them effectively.

If the Wegener’s granulomatosis is targeting your lungs, a doctor might have you take a combination antibiotic to reduce your chances of infection.

Plasma Exchange

Plasma exchange, also called plasmapheresis, takes out the liquid part of your blood, called plasma, that holds disease-producing substances. In the same treatment, you will receive either fresh plasma or a protein, albumin, that helps your body create new plasma. Your doctor will prescribe a plasma exchange if you have a severe case of Wegener’s granulomatosis in order to help your kidneys recover.

What are lifestyle changes that could help with Wegener's granulomatosis?

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Wegener’s granulomatosis is a type of vasculitis. People living with any type of vasculitis can make some lifestyle changes that will help their body recover more easily.

  • Eat a healthy diet. Include fruit, vegetables, lean proteins, and whole grains. Try to avoid sugar and alcohol. You might need to incorporate a vitamin D or calcium supplement if your vasculitis medication impacts your bones.
  • Exercise regularly. Stretching, walking, and other types of low-impact exercise can boost your bone health, blood flow, strength, and mood. Exercise can also improve your mood by reducing stress.
  • Quit smoking. If you need help with quitting, speak with your doctor about strategies and treatments you can use.

Should you see a doctor for Wegener's granulomatosis?

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See a doctor right away if you believe you have Wegener’s granulomatosis. Because this condition can present in so many different ways, you should be on the look-out for abnormal symptoms and make a doctor’s appointment if any arise.

You should see a doctor if you exhibit the following symptoms:

  • Runny nose (does not resolve with over-the-counter cold medicines)
  • Nosebleeds
  • Pus-like material from nose
  • Coughing up blood

Wegener’s granulomatosis gets progressively worse, so it is imperative that you seek treatment right away. A treatment plan and a dedicated care team can help you resolve symptoms.

What is the outlook for people living with Wegener's granulomatosis?

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If you are living with Wegener’s granulomatosis, the outlook depends on the severity of your case and which organs it has affected. Medication can usually treat this disease, but relapses are incredibly common. You will work with your doctor on follow-up visits to make sure the Wegener’s granulomatosis does not return and cause further health complications. To prevent a relapse, you will need to take medication long-term.

Undiagnosed or untreated Wegener’s granulomatosis can progress quickly and produce health complications. Possible complications include:

  • Skin scars
  • Hearing loss
  • Lung failure
  • Kidney failure
  • Heart disease
  • Anemia
  • Nose damage
  • Deep vein thrombosis

Although most people with Wegener’s granulomatosis recover with treatment, you might still have some stress about your condition. You could also feel stressed about relapses and consequent health complications. To cope with your condition, you should:

  • Lean on a support system. Talk with family, friends, or other loved ones about your feelings. You might also benefit from connecting with other people who have Wegener’s granulomatosis.
  • Learn about your condition. Educating yourself about Wegener’s granulomatosis can help you navigate medication side effects, health complications, and relapses. You can speak with your doctor, or even a counselor or medical social worker.