Granulomatosis with Polyangiitis (formerly Wegener’s Granulomatosis)

What is granulomatosis with polyangiitis (GPA)?

Granulomatosis with polyangiitis (GPA) is a type of vasculitis — chronic inflammation in your blood vessels. It was formerly called Wegener’s granulomatosis, but the new name describes it more precisely. GPA involves:

• Inflammation in many different types of blood vessels throughout your body (polyangiitis)
• Inflammatory masses called granulomas forming in your blood vessels and organs (granulomatosis)

Different types of vasculitis affect different blood vessels. GPA affects the many smaller blood vessels throughout your body. This means it can cause issues throughout your body, but especially in organs that rely on many small blood vessels. GPA tends to affect your respiratory system and kidneys the most.

Inflammation in your blood vessels can cause them to swell, break and bleed. It can also cause scarring that restricts the blood flow through your vessels, depriving your tissues of oxygen and nutrients. GPA can cause bleeding and organ damage in your renal and pulmonary systems (pulmonary-renal syndrome).

What are the symptoms of granulomatosis with polyangiitis (Wegener’s granulomatosis)?

GPA affects your respiratory system and kidneys the most. But it can also affect many other organs and tissues, and how you feel overall. The smaller blood vessels that it targets travel throughout your body.

Vasculitis in your connective tissues may cause muscle pain and joint pain, along with an overall feeling of unwellness (malaise). Other general symptoms may include fatigue, loss of appetite and weight loss.

Respiratory symptoms

Many people with GPA first notice and seek treatment for symptoms in their respiratory system. Early symptoms might resemble a cold or infection, but they linger too long. They might include:

• Sinus headaches or pressure
• Persistent sinus infections
• Persistent runny nose, with crusting around your nostrils
• Nasal congestion
• Unexplained, repeated nosebleeds
• Earache
• Chest pain
• Chronic cough
• Shortness of breath

As granulomatosis with polyangiitis worsens, you might develop inflammation of the cartilage in your ears, nose and throat (polychondritis). This can cause more severe symptoms, including:

• Nasal swelling so severe that your nose bridge collapses (saddle nose)
• Narrowing in your throat (subglottic stenosis), causing hoarseness, wheezing or stridor
• Inner ear inflammation, causing vertigo and hearing loss

You can also bleed in your lungs, which may cause shortness of breath or coughing up blood.

Kidney symptoms

You may not experience kidney pain with GPA. But you may notice changes in your pee, like foamy pee or blood in your pee. Swelling in your face or feet (edema) is another symptom of kidney disease.

If you have GPA, healthcare providers will continue to check on your kidneys, even if you never develop symptoms. While kidneys aren’t always involved in GPA early on, they almost always are eventually.

Eye symptoms

GPA can affect your eyes, causing inflammation on the surface or the inner parts of your eye. This can cause eye pain and pressure and make your eyes red and swollen. Severe cases can lead to vision loss.

Skin symptoms

Vasculitis in your skin may show up as:

• Mottled skin, with a bluish-red pattern.
• Purple, red or brown splotches (purpura).
• Skin lesions, like hard nodules or papules.
• Open sores (ulcers).

Nervous system symptoms

Granulomatosis with polyangiitis may affect your nervous system. It usually affects your peripheral nervous system — the nerves that extend from your spinal cord to the rest of your body.

Inflammation and swelling may compress or damage just one nerve, causing symptoms in just one area (mononeuropathy). More commonly, it may affect multiple nerves (polyneuropathy). Symptoms can include:

• Motor symptoms, like muscle weakness
• Sensory symptoms, like numbness and tingling or nerve pain
• Loss of autonomic control of your blood pressure, bladder or sweating

What are the most serious complications of granulomatosis with polyangiitis (Wegener’s)?

Severe GPA can be organ-threatening and life-threatening. Complications can include:

• Bleeding in your lungs (pulmonary hemorrhage), which can lead to respiratory failure
• Rapidly progressive glomerulonephritis (kidney disease), which can lead to kidney failure
• Permanent vision loss or hearing loss
• Loss of skin sensation or muscle control (peripheral neuropathy)

What causes granulomatosis with polyangiitis (GPA)?

GPA, like other types of vasculitis, is an autoimmune disease. This means your immune system generates ongoing inflammation in your blood vessels. Normally, your immune system sends inflammation to target infections. But in autoimmune diseases, it targets your healthy tissues by mistake.

What triggers granulomatosis with polyangiitis?

Researchers aren’t sure why autoimmune diseases occur. There are probably multiple factors involved. Sometimes, extra stress on your immune system seems to push it into overdrive, triggering dysfunction. Researchers have associated the onset of GPA with certain severe bacterial and viral infections.

Granulomatosis with polyangiitis (GPA) belongs to a group of conditions called ANCA-associated vasculitis, or AAV. These are types of vasculitis that involve a specific antibody called ANCA. Researchers believe this antibody plays a role in triggering the inflammation in these conditions, including GPA.

How is granulomatosis with polyangiitis diagnosed?

A healthcare provider will begin by asking about your symptoms and physically examining you. If your symptoms and medical history suggest GPA, they’ll follow up with tests to investigate further.

Tests for granulomatosis with polyangiitis include:

• Blood tests, including testing for ANCA antibodies
• Urinalysis, including testing your urine for blood and protein
• Chest X-ray and CT scans to look at your lungs
• Biopsy of lung, sinus or kidney tissue to look for specific types of inflammation

What is the treatment for granulomatosis with polyangiitis (Wegener’s)?

Healthcare providers treat granulomatosis with polyangiitis (Wegener’s) with anti-inflammatory and immune system-suppressing drugs (corticosteroids and other immunosuppressants).

Some of these medications are stronger than others. Which combination your provider prescribes will depend on how severe your condition is and how much you’re at risk of the side effects.

Initial treatment for active GPA includes:

• High doses of corticosteroids, such as prednisone. These doses will eventually taper off.
• Rituximab. This newer biologic medication is now standard treatment.
• Cyclophosphamide (for severe disease) or methotrexate (for milder disease). These drugs also treat cancer, but for GPA (at much lower doses), they function as immunosuppressants.
• Avacopan. This drug can be used as an adjuvant treatment to reduce your dosage of corticosteroids.

If you have severe complications, you might need additional treatment, such as:

• Dialysis
• Organ transplantation

Once GPA is in remission, you’ll take milder medications to maintain remission. These typically include:

• Rituximab
• Milder immunosuppressants, such as azathioprine, methotrexate or mycophenolate mofetil

You’ll have ongoing testing during treatment to monitor your condition and response to the treatment. Your healthcare provider will adjust your prescription and dosage according to your response.

Each medication comes with its own potential side effects, and they can occur at any time during your treatment. You might need to change medications to avoid or reduce the risk of certain side effects.

What is the outlook for this condition?

Granulomatosis with polyangiitis is a lifelong condition that requires lifelong care. With treatment, the disease can go into remission and stop causing symptoms. But symptoms can also return (relapse).

You may have to take medications off and on for the rest of your life to manage relapses. Since these medications suppress your immune system, you’ll also have to take extra care to prevent getting sick.

Can you live a normal life with GPA?

With effective treatment, you can live a relatively normal life with GPA. Most people with chronic diseases have periods of remission and relapse, so the course of the disease can be up and down.

What is the life expectancy of someone with granulomatosis with polyangiitis?

Without treatment, the average life expectancy is five months, with less than 50% surviving one year. But with treatment, follow-up studies show more than 80% of people are alive at least eight years later.

What is the most common cause of death in granulomatosis with polyangiitis?

Before treatment, organ failure is the biggest mortality risk with GPA. During treatment, you’re more at risk of life-threatening infections that can take hold when your immune system is suppressed.

How should I take care of myself while living with granulomatosis with polyangiitis?

When you’re living with a chronic autoimmune disease like granulomatosis with polyangiitis, you need to maintain a certain awareness of your body. Even when you’re feeling well, you should live defensively.

Take steps to protect yourself from common illnesses while taking immunosuppressants. Make sure to notice any new or unusual symptoms and let your healthcare provider know about them right away.