In a recent study published in Clinical and Experimental Immunology, researchers determined that patients with high ANCA antibody levels are at an increased risk of having ANCA-associated vasculitis (AAV) relapses. According to ANCA Vasculitis News, these high ANCA antibody levels may also contribute to a loss of kidney function. Ultimately, researchers determine that more aggressive treatment options might be needed for these patients.

ANCA Antibodies

The antibodies on the attack in AAV are anti-neutrophil cytoplasmic autoantibodies (ANCAs), which attack proteinase 3 (PR3) or bind to the MPO protein. While there is no common monitoring system following AAV diagnosis, many doctors still make sure to frequently check a patient’s ANCA antibody levels.

In the recent study, researchers wanted to understand how this follow-up contributed to patient health, particularly relapses and kidney failure. 99 patients enrolled. In the follow-up after their diagnosis, patients were tested at least 5 times. Of the patients, 8 had eosinophilic granulomatosis with polyangiitis (EGPA), 36 had granulomatosis with polyangiitis (GPA), and 55 had microscopic polyangiitis (MPA). 67 patients had MPO-ANCA antibodies. 32 patients had PR3-ANCA antibodies.

Patients with AAV who were considered recurrent (periods of ANCA antibodies followed by times of negative results) and persistent (patients who continued having high levels of ANCA antibodies) relapsed at a rate much higher than those in other groups. Patients with arthritis, and patients taking Rituximab, were at a higher risk of having a relapse. However, patients with arthritis were less likely to experience kidney failure.

Overall, researchers determined that patients with recurrent or persistent AAV with high levels of ANCA antibodies are more at risk of relapse and might require more aggressive therapies.

ANCA-Associated Vasculitis

According to the Genetic and Rare Diseases Information Center (GARD):

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of diseases characterized by destruction and inflammation of small [blood] vessels.

The diseases that make up ANCA-associated vasculitis include granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis.

Granulomatosis with Polyangiitis (GPA)

Once known as Wegener’s granulomatosis, granulomatosis with polyangiitis (GPA) is a rare blood vessel disease which causes blood vessel inflammation throughout the body. While doctors are not sure of the exact cause, some believe GPA might result from an infection or similar trigger. Generally, GPA affects the sinuses, lungs, kidneys, eyes, skin, nerves, ears, and joints. Symptoms include:

• Coughing up blood
• Difficulty breathing
• Congestion
• Fatigue
• Fever
• Night sweats
• Muscle numbness
• Joint pain
• Difficulty hearing
• Rashes
• Frequent nosebleeds
• Organ failure

Eosinophilic Granulomatosis with Polyangiitis (EGPA)

The Johns Hopkins Vasculitis Center describes eosinophilic granulomatosis with polyangiitis (EGPA) as:

a systemic vasculitis. This disease was first described in 1951 by Dr. Jacob Churg and Dr. Lotte Strauss as a syndrome consisting of “asthma, eosinophilia [an excessive number of eosinophils in the blood], fever, and accompanying vasculitis of various organ systems.”

Symptoms include:

• Asthma
• High eosinophil (a type of white blood cell) levels
• Coughing up blood
• Nasal polyps
• Allergies
• Kidney inflammation

Microscopic Polyangiitis (MPA)

There is no known cause for microscopic polyangitis, although some researchers consider it an autoimmune disorder. This rare condition is characterized by blood vessel inflammation, particularly in the kidneys, skin, joints, nervous system, and lungs. As the blood vessels weaken and narrow, it heightens the risk of aneurysms and blood vessel rupturing. MPA can negatively impact organ function and even cause organ failure. Symptoms vary but may include:

• Skin rashes
• Unintended weight loss
• Appetite loss
• Fatigue
• Fever
• Muscle and joint pain
• Loss of mobility
• Coughing up blood
• Difficulty breathing
• Lung disease and respiratory failure
• Bloody urine
• Kidney inflammation and damage