According to a story from Medscape, a new set of guidelines from the Kidney Disease: Improving Global Outcomes (KDIGO) aims to improve and update standards of care for antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis. These new guidelines are intended to take into account some of the latest developments in the treatment of ANCA-associated vasculitis, a rare disease.

Perhaps the most pressing of these new developments is the approval of avacopan, a medication of the C5a receptor inhibitor class. Avacopan was approved as an add-on treatment to be used in conjunction with standard of care. The guidelines include info on the use of this therapy as an alternative option to glucocorticoids. They also account for recent results on the use of lower dose corticosteroids. The guidelines also recommend plasma exchange in patients dealing with advanced kidney disease.

Other important updates to the treatment guidelines include:

• A quicker reduction of glucocorticoids when used in combination with rituximab or cyclophosphamide during induction therapy.
• Patients most likely to benefit from the use of avacopan have a higher risk of toxicity from glucocorticoids.
• Superior recovery of glomerular filtration rate (GFR) in avacopan vs glucocorticoids.
• Recommendation of plasma exchange in patients experiencing anti-glomerular basement membrane disease.

About ANCA-Associated Vasculitis

ANCA-associated vasculitis is a group of diseases that are characterized by the damage and destruction of blood vessels as a result of inflammatory activity. The disease is associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). These are autoantibodies that target antigens present on neutrophils (the most common type of white blood cell) and monocytes. This means that the mechanism of the disease is autoimmune, in which the body’s own immune system mistakenly attacks healthy body tissue. Symptoms of ANCA associated vasculitis include kidney inflammation, fever, weight loss, abdominal pain, bloody stools, purpura, nose bleeds, muscle pain, arthritis, bloody cough, vision problems, headaches, stroke, heart attack, and high blood pressure. Treatment of the disease is primarily focused on controlling inflammation and suppressing immune system activity. Common medications include cyclophosphamide, rituximab, and prednisone. Antibiotics may be necessary in cases of infection. To learn more about ANCA- associated vasculitis, click here.