Pulmonary fibrosis—an uncommon manifestation of anti-myeloperoxidase-positive systemic vasculitis?

Small vessel vasculitides such as microscopic polyangiitis and Wegener’s granulomatosis commonly involve the kidney and lung, with alveolar haemorrhage being the commonest manifestation of pulmonary involvement. Here we describe a patient who developed acute renal failure and pulmonary haemorrhage with positive autoantibodies against myeloperoxidase 1 year after a diagnosis of usual interstitial pneumonia had been made and we discuss the uncommon association of pulmonary fibrosis and anti-myeloperoxidase positive vasculitis.