ANCA-Associated Vasculitis: What is it?

Kidney Disease Specialist Barry I. Freedman, MDBarry I. Freedman, MD, chief of Nephrology, is a clinician researcher who focuses on genetic factors in kidney disease.

A kidney disease condition called ANCA-associated vasculitis made headlines when Wake Forest University student-athlete Kevin Jordan suffered from the disease and needed a kidney transplant. Wake Forest's head baseball coach Tom Walter donated one of his kidneys on Feb. 7, 2011 in a successful surgery at Emory University Hospital in Atlanta.

As Dr. Freedman explains, ANCA vasculitis (named for Anti-Neutrophil Cytoplasmic Antibody) is an example of an autoimmune disease that can affect blood vessels throughout the body. “The kidney is effectively composed of blood vessels and they can be severely affected by this disorder,” he said.

Normal antibodies are produced by the immune system and circulate in the blood stream to fight infection. However, abnormal autoantibodies, like ANCA, can attack a person’s own cells and tissues, specifically white blood cells which are called neutrophils. These neutrophils damage the walls of small blood vessels in different organs, a syndrome called "vasculitis" or blood vessel inflammation. When this occurs in the kidneys, it causes blood and protein to leak into the urine, and can lead to kidney failure.

“If you don’t aggressively treat ANCA vasculitis early on, it often progresses to end-stage kidney disease requiring dialysis or a kidney transplant,” Freedman said.

 

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