The pulmonary vasculitides are a rare category of diseases characterised by inflammation and necrosis of the blood vessel wall, mainly involving small and medium-sized blood capillaries in the lung. Management has typically included the use of immunosuppressive medications as well as newer adjuvant therapies such as plasmapheresis. Recent trials over the last decade have emphasised the effective use of these medicines in treating this life-threatening condition; nevertheless, much remains unclear in terms of exact dosage of these agents and length of treatment. Adverse effects from these treatments continue to be a major issue, necessitating thorough monitoring and a multidisciplinary approach.

Exploration of novel medicines, some of which are now being utilised in randomised trials, is another advancement in this discipline. This study provides an update on current developments in the therapy of pulmonary vasculitis, as well as a proposed consistent strategy to management based on disease severity categorization.