Purpose of Review <p>Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides, particularly granulomatosis with polyangiitis and microscopic polyangiitis, are rare but severe systemic diseases frequently requiring intensive care unit (ICU) admission due to life-threatening renal and pulmonary involvement. Despite advances in immunosuppressive therapy, these patients remain at high risk of mortality and end-stage kidney disease, with prognosis driven by organ failure (notably diffuse alveolar haemorrhage [DAH]) and treatment-related complications such as infection. ICU management is complex and relies on rapid diagnosis, aggressive induction therapy, and tailored organ support.</p> Recent Findings <p>Current remission-induction strategies centre on high-dose glucocorticoids combined with cyclophosphamide or rituximab, with growing emphasis on minimizing steroid exposure. Plasma exchange (PLEX), once widely used for severe renal disease and DAH, remains debated, although selected high-risk subgroups may still derive benefit.</p> Summary <p>Optimal management in the ICU requires a multidisciplinary approach, including careful selection of immunosuppressive regimens, individualized use of adjunctive therapies such as PLEX and the management of infectious complications. Advances in supportive care, including lung-protective ventilation, renal replacement therapy, and extracorporeal support, have improved short-term outcomes. However, significant uncertainty persists regarding optimal therapeutic strategies in the critically ill populations, highlighting the need for further research and phenotype-driven approaches.</p>