Abstract
In patients with anti-neutrophil cytoplasm antibody-associated vasculitides, emergency situations can arise from either life- or organ-threatening manifestations of the underlying disease or from complications associated with immunosuppressive treatment, such as severe infections. Among the most severe manifestations of AAV, diffuse alveolar haemorrhage and severe gastrointestinal manifestations are immediately life-threatening and require immediate diagnosis and treatment. Patients with these manifestations should be monitored and treated in an intensive care unit. Further manifestations of AAV are rapid progressive glomerulonephritis, involvement of the central nervous system, subglottic stenosis, cardiac manifestations and thrombembolic events. This review is aimed to focus on the diagnosis, treatment and prognosis of these severe manifestations of the AAVs.
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