Vasculitis in the seriously ill patient: diagnostic approaches and therapeutic options in ANCA-associated vasculitis.

The clinical severity of ANCA-associated vasculitides (AAV) ranges widely from relatively benign courses to rapidly progressive, acutely life-threatening disease. The aggressiveness in the individual patient depends on the site and size of vessels involved and on the severity of the associated inflammatory/immunological features. The scope of life-threatening lesions reaches from the well-known pulmonary renal syndrome to cardiac, gastrointestinal and central nervous system manifestations. Treatment must be instituted as soon as possible and consists of daily cyclophosphamide and high-dose glucocorticosteroids. Plasmapheresis, intravenous immunoglobulin, and monoclonal antibodies may be of benefit in some cases.