‘Early detection of ANCA-associated vasculitis can stop dialysis and prevent lifelong treatment’

It’s often believed that once a person’s kidneys fail and they start dialysis, they’ll remain on it for life -- or won’t come off it unless they receive a kidney transplant. But that’s not always the case. A rare autoimmune disease called ANCA-associated vasculitis can cause kidney failure, and if detected and treated early -- before the vasculitis causes irreversible damage -- it may be possible to stop dialysis.

ANCA-associated vasculitis is a type of immune dysregulation in which antibodies known as antineutrophil cytoplasmic antibodies (ANCAs) target the walls of the body's small blood vessels, or capillaries. This attack causes inflammation that can damage various organs, including the skin, kidneys, lungs, nervous system, cardiovascular system, and peripheral nerves.

ANCA-associated vasculitis can become so severe that patients may require kidney dialysis -- but if diagnosed early, they can live without ever needing it. “The idea is that if the inflammation is so severe that the blood vessels are on fire, you can put it out before it burns down all,” said Lee Sang-won, professor of rheumatology at Severance Hospital.

For patients with ANCA-associated vasculitis who are on kidney dialysis, current guidelines are unclear about when dialysis can be stopped. However, Lee said, “Patients with ANCA-associated vasculitis who have had the disease for less than two months have some chance of returning to function. This seems to be true for blood vessels in all organs, not just the kidneys.”

ANCA-associated vasculitis is difficult to diagnose early because it primarily affects microvessels and can involve the entire body, leading to a wide range of symptoms. However, if diagnosed and treated in time, it can be a manageable condition that does not become fatal.

“Because ANCA-associated vasculitis primarily involves capillaries, it can affect the entire body and cause a wide range of symptoms,” said Lee. “Early detection is the most important key to treating ANCA-associated vasculitis, as it can minimize organ damage caused by the condition.”

The most commonly affected organ in ANCA-associated vasculitis is the skin -- because it contains the largest number of capillaries.

“The most common symptom of ANCA-associated vasculitis is a rash on the skin. There are many patients who visit a dermatologist and are diagnosed with systemic vasculitis -- a condition that has already spread throughout the body,” said Lee.

After the skin, the lungs and kidneys are the next most commonly affected organs in ANCA-associated vasculitis.

Treatment for ANCA-associated vasculitis is immunosuppressive and includes steroids, immunosuppressive drugs, and monoclonal antibody drugs targeting B cells.

“Since the cause of ANCA-associated vasculitis is mainly an immune abnormality, we do not use immune-enhancing treatments, but rather treatments that slightly suppress the immune system. Steroids are the most common drugs, and many other immunosuppressive treatments can be used to control vasculitis early,” Lee said.

ANCA-associated vasculitis is not curable and requires lifelong treatment and management.

“We can't completely rebuild our body's immunity, so it stays with us as long as we live. A state in which the disease is present but not showing symptoms is called 'remission,' and the goal of treating ANCA-associated vasculitis is to achieve remission quickly and maintain it for life,” Lee said.