A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels () leading to damage in any number of organs. The common features include granulomatous inflammation of the and kidneys. Most patients have measurable autoantibodies () against neutrophil proteinase-3 ().
Angéite granulomateuse nécrosante /
Maladie de Wegener /
Syndrome de Wegener /