ANCA-Associated Vasculitis: A Silent but Serious Disease

Vasculitis is a group of rare disorders where the body’s own immune system attacks and inflames blood vessels. One important type is ANCA-associated vasculitis (AAV), which can affect small to medium-sized vessels throughout the body. If untreated, it can damage vital organs such as the kidneys, lungs, nerves, skin, and sinuses.

What is ANCA?

ANCA stands for Anti-Neutrophil Cytoplasmic Antibody, a type of autoantibody that mistakenly targets a person’s white blood cells. These activated white cells then injure the walls of blood vessels, causing inflammation and narrowing that reduce blood flow to tissues.

Who can be affected?

AAV can occur at any age but is most common in people over 40. Both men and women can be affected. The disease often develops slowly, beginning with vague symptoms such as fatigue, fever, weight loss, or joint pains, before progressing to organ involvement.

Symptoms to watch for

• Kidneys: blood or protein in urine, kidney failure.
• Lungs: cough, shortness of breath, coughing up blood.
• Sinuses, nose and ear: persistent congestion, nosebleeds, ear discharge, hearing loss
• Nerves: numbness, weakness.
• Skin: rashes, purplish spots.

Because the symptoms vary widely, diagnosis is often delayed. Blood tests (including ANCA), urine test, imaging (XRAY or CT scan of the chest), and sometimes tissue biopsy are used to confirm the condition.

Treatment and outlook

Treatment has improved remarkably over the last two decades. The treatment happens in 2 parts, 1 after the other. It involves medicine that suppresses the body’s infection-fighting system, called the immune system.

The first part of treatment is called “induction”. Patients will need to take strong medicines such as corticosteroids and immunosuppressive agents (cyclophosphamide or rituximab) to bring the disease under control. The second part of treatment is called maintenance. For this part, doctors will switch to a lower dose of medicine that helps prevent relapses. With timely treatment, many patients achieve remission, though long-term follow-up is essential