Pulmonary haemosiderosis is characterised by chronic alveolar haemorrhage, which can lead to serious cardiorespiratory complications. Although considered idiopathic in most patients, there are many possible aetiologies. We present a case of an 18-year-old woman with pulmonary haemosiderosis since 4 years of age, with an inconclusive initial study, who was treated with systemic corticosteroids and hydroxychloroquine until the age of 12 years, and azathioprine since then. Multiple exacerbations led to interstitial lung disease with restrictive functional pattern. Unilateral cochlear deafness was diagnosed at the age of 12 years and occasional polyarthralgias were recorded. When she was 16 years of age the study revealed an atypical myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) pattern. Cyclophosphamide and rituximab were administered with resolution of respiratory insufficiency and functional disability, without new episodes of alveolar haemorrhage. This case of chronic pulmonary haemorrhage was revealed to be an ANCA vasculitis, the diagnosis of which was possible only after 12 years of symptoms, with clinical and functional improvement with the association of cyclophosphamide and rituximab.
2015 BMJ Publishing Group Ltd.