ANCA-Associated Vasculitis

Immune & Autoimmune

Overview
Symptoms
Resources
Stories & Profiles

Overview

ANCA-associated vasculitis, or AAV, is a group of rare diseases where the immune system attacks small and medium blood vessels by mistake. This causes the vessel walls to swell and get thick, which makes it harder for blood to flow through. Over time, this can hurt organs and tissues that depend on that blood flow.

“ANCA” stands for anti-neutrophil cytoplasmic antibodies. These are special proteins made by the immune system that attach to certain white blood cells. When this happens, the white blood cells can attack blood vessels instead of germs.

AAV is serious, but it is treatable. Most people need strong medicine at first to calm the disease, then longer term treatment to keep it quiet. Many people with AAV live active lives while working closely with their care team.

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At A Glance

Autoimmune condition Affects small blood vessels Can involve multiple organs Requires specialist care

Common Signs And Symptoms

Fatigue
Difficulty With Daily Activities Climbing Stairs Lifting Objects Rising From A Chair
Shortness Of Breath
Dry Eyes Mouth And Nose
Shortness Of Breath And Chest Pain
Lung Issues Shortness Of Breath Coughing Lung Fibrosis
Bloody Or Foamy Urine
High Blood Pressure
Pinhead Sized Red Spots On The Skin Petechiae
Tingling And Numbness In The Limbs

Types of ANCA-Associated Vasculitis

AAV is an umbrella term for three main types:

GPA(Granulomatosis with polyangiitis)Often affects the sinuses, nose, ears, lungs, and kidneys. People may notice long-lasting sinus problems, nosebleeds, ear issues, or coughing and shortness of breath.
MPA(Microscopic polyangiitis)Often involves the kidneys and lungs, and can also affect nerves, skin, and joints. It may show up as blood or protein in the urine, high blood pressure, shortness of breath, or a cough with blood.
EGPA(Eosinophilic granulomatosis with polyangiitis)Usually happens in people with asthma and allergies. It can affect the lungs, nerves, skin, and other organs. People may notice asthma that gets worse, nerve pain or weakness, or rashes.

Doctors may also describe AAV by the type of ANCA in the blood, such as PR3-ANCA or MPO-ANCA, because these can be linked with different patterns of disease.

Diagnosis Journey

First visitYou may start with a primary care doctor because you feel very tired, have sinus problems, a rash, or shortness of breath.Referral to a specialistIf your doctor is worried about your kidneys, lungs, nerves, or immune system, they may send you to a specialist, such as a rheumatologist, nephrologist, pulmonologist, or neurologist.Blood and urine testsANCA blood test, to look for the antibodies that give AAV its name; Kidney tests and urine tests, to check for blood or protein; Basic labs, to check for inflammation and organ function.Imaging testsChest X-ray or CT scan, to look at your lungs; Sinus imaging, to look at your nose and sinuses.BiopsyIn many cases, doctors try to take a tiny piece of tissue from an affected organ, such as the kidney, skin, lung, or nerve. A pathologist looks at it under a microscope to see signs of vasculitis.Diagnosis and planYour care team reviews all the results and decides if you have AAV and which type. Then they create a treatment plan that fits your level of disease and which organs are involved.

Treatment Options

Treatment usually has two main phases:

01Induction treatmentto get the disease under control.
02Maintenance treatmentto keep it quiet and prevent flares.

Research & Resources

Clinical research helps scientists learn more about ANCA-Associated Vasculitis and develop better treatments for the future.

FEATURED CLINICAL STUDY

AVASET Clinical Study

A clinical study for adults with newly diagnosed or relapsed ANCA-associated vasculitis, including granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). If you have GPA or MPA, you may be eligible for this study, which is evaluating a study drug.

Recruiting

View Study

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Vasculitis FoundationLearn More