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Keywords:

  • antineutrophil cytoplasmic antibodies (ANCA);
  • ANCA-associated vasculitis (AAV);
  • microscopic polyangiitis;
  • vasculitis;
  • Wegener's granulomatosis

Abstract

Antineutrophil cytoplasmic antibody-associated vasculitis is an autoimmune disease involving small to medium blood vessels. It is an uncommon illness, but can have devastating consequences, particularly on kidney function and other vital organs. Exciting progress has been made in the treatment of the disease largely because of international collaboration in randomised clinical trials. Patient survival has improved dramatically with advancements in disease diagnosis and medical treatment. The long-term morbidity from the disease, although improving, remains substantial with up to 10% of survivors requiring dialysis or kidney transplantation. Clinical trials are underway using more specifically targeted immunosuppressants in the hope to improve the long-term patient outcomes. Advancements are also being made in understanding the pathogenesis of the disease and this will further assist disease treatment and outcomes in the future.