ANCA-Associated Vasculitis

ANCA vasculitis is a group of rare autoimmune diseases that cause inflammation and damage in small and medium blood vessels. Healthcare providers also call it ANCA-associated vasculitis or AAV.

ANCA is an abbreviation for antineutrophil cytoplasmic antibodies. Antibodies are proteins your immune system makes to identify and destroy invaders like germs. Autoantibodies are proteins that mistakenly target healthy tissue.

If you have one of these diseases, your immune system damages blood vessels and organs throughout your body. They can affect your:

• Kidneys
• Lungs and other parts of your respiratory system
• Ears, nose and sinuses
• Skin
• Nerves
• Joints
• Heart

The inflammation in your blood vessels makes them swell and thicken. This makes it harder for blood to flow through them like it should. Over time, the inflammation damages the blood vessels and the tissue they’re connected to.

Types of ANCA vasculitis

There are three types of ANCA-associated vasculitis:

• Eosinophilic granulomatosis with polyangiitis (EGPA)
• Granulomatosis with polyangiitis (GPA)
• Microscopic polyangiitis (MPA)

ANCA-associated vasculitis symptoms

The symptoms can vary. What you experience depends on which type you have and where it affects you. Some ANCA-associated vasculitis symptoms include:

• Fever
• Fatigue
• Feeling sick or generally unwell
• Unexplained weight loss or loss of appetite
• Skin rashes or discolored patches of skin
• Shortness of breath or trouble breathing
• Coughing and/or coughing up blood
• Pain in your muscles, joints, eyes, nose, sinuses or chest
• Heart palpitations
• Muscle weakness
• Numbness or tingling
• Growths called polyps inside your nose
• Blood in your pee or poop

There are lots of symptoms. And some of them can feel like minor, everyday issues. The best thing you can do is trust yourself. You know when something’s unusual or not quite right. Visit a healthcare provider if you’re experiencing any new symptoms that make you feel worried.

ANCA-associated vasculitis causes

ANCA vasculitis is an autoimmune disease. This happens when your immune system damages your body instead of protecting it. Usually, your body uses inflammation to help you heal. But if you have ANCA-associated vasculitis, your immune system creates inflammation in blood vessels that don’t need it. This leads to damage and symptoms.

Experts aren’t sure what makes your body start damaging itself. Most people with ANCA vasculitis have the antibodies in their blood. Some researchers think having these antibodies can trigger the diseases. But they can’t say this for certain, because not everyone has them. And some people have the antibodies but never develop vasculitis.

Risk factors

Anyone can develop these conditions. But they usually develop in people between age 40 and 50. Males have a slightly higher risk.

Complications of this condition

These diseases can cause serious, potentially life-threatening complications if they damage organs like your lungs, kidneys or heart. Severe ANCA-associated vasculitis can cause:

• Bleeding in your lungs
• Lung scarring
• Nerve damage
• Kidney failure
• Heart failure

How doctors diagnose antineutrophil cytoplasmic antibody-associated vasculitis

A healthcare provider will diagnose ANCA vasculitis with a physical exam, questions about the symptoms you’ve experienced and by doing some tests. Tell your provider which symptoms you’ve had, and if they seem to come and go. Let them know if any activities or times of day seem to make symptoms worse.

Diagnosing vasculitis is usually part of a differential diagnosis. This means your provider will rule out other, more common conditions first. You might need a few different tests, including:

• Blood tests (especially an ANCA test)
• Urinalysis
• Chest X-ray
• MRI
• CT scan
• Angiograms

You’ll work with a rheumatologist. This is a healthcare provider who specializes in treating inflammatory conditions like vasculitis. You may need to meet with other specialists if it affects your organs.

ANCA-associated vasculitis treatment

Preventing organ damage and managing the inflammation are the most important parts of treating these conditions. Your healthcare provider will prescribe medications to control inflammation and calm down your immune system. The most common options include:

• Corticosteroids: These are prescription anti-inflammatory medications. They can also suppress your immune system.
• Rituximab: This medication targets antibodies and slows down your immune system. Your provider will usually prescribe it alongside corticosteroids.
• Cyclophosphamide: This is a medication that’s usually part of chemotherapy to treat some cancers. Providers prescribe it in lower doses to manage vasculitis. This is another option your provider may suggest in addition to corticosteroids.
• Other immunosuppressants: Your provider might suggest other medications that reduce your immune system’s activity.

Your provider will probably start you on a relatively high dose of medication. This will help you get into remission. Remission is a period where you have little to no inflammation in your body. Once you’re in remission, they’ll adjust the medications to help keep you there as long as possible.

Each type of ANCA vasculitis may need slightly different treatments. What’s best for you will depend on which type you have, where it affects you and its severity. Your provider will tell you what you’ll need and what to expect based on your unique situation.

When should I see my healthcare provider?

Your provider will tell you how often you’ll need regular follow-up appointments and tests.

Talk to your provider if you notice any changes in the symptoms. Especially if it feels like they’re more severe or happening more often. Tell your provider if it feels like your treatments aren’t working as well as they used to.

When should I go to the emergency room?

Go to the emergency room or call 911 (or your local emergency number) if you:

• Cough up blood
• Feel like you can’t breathe
• Have any other new symptoms that concern you
• Have a fever

What can I expect if I have ANCA-associated vasculitis?

You should plan to manage ANCA vasculitis for a long time. Maybe the rest of your life. There’s no cure for it. But once you enter remission, you should be able to avoid having symptoms for a long time. Some people are in remission for months or years at a time.

But there’s always a chance symptoms can come back. It’s especially common in people with GPA. These conditions affect everyone differently. Talk to your healthcare provider about what to expect.