Case of the Month #25 - ANCA vasculitis

Published 01/02/2022

What is the management of ANCA vasculitis?

Specialist referral is essential for management. Typically this would be the renal team or respiratory team if pulmonary haemorrhage is the primary feature. Initial management is high dose methylprednisolone, followed by immunosuppression induction therapy in the form of cyclophosphamide or Rituximab. Plasma exchange had previously been widely used, but this has recently been shown to not reduce the risk of death or end stage kidney disease in ANCA vasculitis in a large randomised controlled trial1. Questions remain about plasma exchange in ANCA associated pulmonary haemorrhage and specialist opinion should be sought.

Management of pulmonary haemorrhage involves aggressive immunosuppression and supportive care whilst treatment takes effect. Mechanical ventilation can be required and in some cases this can progress to a need for ECMO.

Volume overload should be avoided as this a can worsen alveolar haemorrhage.