Bronchiectasis has previously been reported in two retrospective single centre studies from France and Japan to be associated with microscopic polyangiitis and anti-myeloperoxidase ANCA-associated vasculitis (AAV) [19, 20], and there are reports of people with bronchiectasis and an atypical p-ANCA directed against bactericidal/permeability-increasing protein ANCA, often after Pseudomonas aeruginosa infection of the lungs [21–23]. This evidence concerns the gene BPI and microscopic polyangiitis.