ABSTRACT
Acute management includes treating her hypoxia with oxygen. If hypoxia does not improve, intubation may be necessary. She also has acute renal failure with hyperkalaemia and acidosis. Both are life-threatening complications and she should be managed in a high-dependency or intensive-care setting. Untreated Wegener’s granulomatosis has a poor prognosis with median survival of only five months. Early involvement of a renal specialist is important to diagnose and treat the underlying condition. First-line therapy is similar to treating Goodpasture’s syndrome with pulmonary involvement and includes pulsed methylprednisolone (followed by oral prednisolone) and cyclophosphamide. Plasmapheresis is also required if there is severe organ involvement (respiratory failure requiring ventilatory support, creatinine >354 mmol/L or not responding to first-line therapy). Second-line agents include mycophenolate mofetil, rituximab or leflunamide.
