Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitides in Childhood

Vasculitis is inflammation of the blood vessels and a rare disease. Vasculitis can occur as an independent clinical entity with no known triggers (primary vasculitis). However, vasculitis can also occur as a consequence of another disease or as a side effect of medication (secondary vasculitis). As there are blood vessels all over the body, in principle, all the organs can be affected by vasculitis. This also explains the diverse complaints. A distinction is made between vasculitis of large, medium-sized, and small blood vessels. Some primary vasculitides of small blood vessels are caused by a special form of auto-antibodies (anti-neutrophil cytoplasmic antibodies, ANCA). These antibodies are formed by mature B cells of the immune system. These are called ANCA-associated vasculitides or ANCA-positive vasculitides..